Unusual Cancers of Childhood Treatment (PDQ®)

Last modified: 2016-08-10
Last downloaded: 2016-09-19

   

General Information About Unusual Cancers of Childhood

   

Unusual cancers of childhood are cancers rarely seen in children.

Cancer in children and adolescents is rare. Since 1975, the number of new cases of childhood cancer has slowly increased. Since 1975, the number of deaths from childhood cancer has decreased by more than half.

Unusual cancers are so rare that most children's hospitals are likely to see less than a handful of some types in several years. Because the unusual cancers are so rare, there is not a lot of information about what treatment works best. A child's treatment is often based on what has been learned from treating other children. Sometimes, information is available only from reports of the diagnosis, treatment, and follow-up of one child or a small group of children who were given the same type of treatment.

Many different cancers are covered in this summary. They are grouped by where they are found in the body.

   

Tests are used to detect (find), diagnose, and stage unusual cancers of childhood.

Tests are done to detect, diagnose, and stage cancer. The tests used depend on the type of cancer. After cancer is diagnosed, tests are done to find out if cancer cells have spread from where the cancer began to other parts of the body. The process used to find out if cancer cells have spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan the best treatment.

The following tests and procedures may be used to detect, diagnose, and stage cancer:

Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.

Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.

Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.

Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.

Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.

Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.

Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

Upper endoscopy. A thin, lighted tube is inserted through the mouth to look for abnormal areas in the esophagus, stomach, and first part of the small intestine.

Upper endoscopy. A thin, lighted tube is inserted through the mouth to look for abnormal areas in the esophagus, stomach, and first part of the small intestine.

Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.

Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • X-ray : An x-ray is a type of energy beam that can go through the body and onto film.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
  • PET scan (positron emission tomography scan): A procedure to find malignanttumor cells in the body. A small amount of radioactiveglucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet and radio waves to make a series of detailed pictures of areas inside the body. The pictures are made by a computer. This procedure is also called nuclear magnetic resonance imaging (NMRI).Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
  • Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. Upper endoscopy. A thin, lighted tube is inserted through the mouth to look for abnormal areas in the esophagus, stomach, and first part of the small intestine.
  • Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
  • Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are many different types of biopsy procedures. The most common types include the following:
   

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
   

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if thyroid cancer spreads to the lung, the cancer cells in the lung are actually thyroid cancer cells. The disease is metastatic thyroid cancer, not lung cancer.


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Treatment Option Overview

   

There are different types of treatment for children with unusual cancers.

Different types of treatments are available for children with cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

   

Children with unusual cancers should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatrichealth care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include the following specialists:

   

Seven types of standard treatment are used:

Surgery 

Surgery is a procedure used to find out whether cancer is present, to remove cancer from the body, or to repair a body part. Palliative surgery is done to relieve symptoms caused by cancer. Surgery is also called an operation.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy 

Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are different types of radiation therapy:

The way the radiation therapy is given depends on the type of cancer being treated.

Chemotherapy 

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can affect cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, a body cavity such as the abdomen, or an organ, the drugs mainly affect cancer cells in those areas. Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Hormone therapy 

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances that are made by glands in the body and flow through the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Hormone therapy with drugs called corticosteroids may be used to treat thymoma or thymic carcinoma.

Immunotherapy 

Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy.

  • Interferon: Interferon affects the division of cancer cells and can slow tumor growth. It is used to treat nasopharyngeal cancer and papillomatosis.
  • Epstein-Barr virus (EBV)-specific cytotoxic T-lymphocytes: White blood cells (T-lymphocytes) are treated in the laboratory with Epstein-Barr virus and then given to the patient to stimulate the immune system and fight cancer. EBV-specific cytotoxic T-lymphocytes are being studied for the treatment of nasopharyngeal cancer.
  • Vaccine therapy: Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is used to treat papillomatosis.

Watchful waiting  

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Watchful waiting may be used when the tumor is slow-growing or when it is possible the tumor may disappear without treatment.

Targeted therapy 

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.

   

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Gene therapy 

Gene therapy is a treatment in which foreigngenetic material (DNA or RNA) is inserted into a person's cells to prevent or fight disease. Gene therapy is being studied in the treatment of papillomatosis.

   

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

   

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials is available from the NCI website.

   

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

   

Some cancers and cancer treatments cause side effects months or years after treatment has ended.

Some cancers and cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects may include the following:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some cancers and cancer treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).


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Unusual Cancers of the Head and Neck

Nasopharyngeal Cancer

Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the lining of the nasalcavity (inside of the nose) and throat. It is rare in children younger than 10 and more common in adolescents.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of nasopharyngeal cancer is greatly increased by having an infection with the Epstein-Barr virus (EBV), which infects cells of the immune system. The risk of nasopharyngeal cancer is also increased by having a certain marker on cells.

Nasopharyngeal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Painless lumps in the neck.
  • Nosebleeds.
  • Blocked or stuffy nose.
  • Headache.
  • Pain in the ear.
  • Ear infection.
  • Problems moving the jaw.
  • Hearing loss.
  • Double vision.

Other conditions that are not nasopharyngeal cancer may cause these same signs and symptoms.

When nasopharyngeal is diagnosed, it usually has already spread to lymph nodes in the neck and bones of the skull. It may also spread to the nose, mouth, throat, bones, lung, and/or liver.

Tests to diagnose and stage nasopharyngeal cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose or stage nasopharyngeal cancer include the following:

  • Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • Nasoscopy : A procedure in which a doctor inserts a nasoscope (a thin, lighted tube) into the patient’s nose to look for abnormal areas.
  • Epstein-Barr virus (EBV) tests: Blood tests to check for antibodies to the Epstein-Barr virus and DNA markers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV.

Prognosis

The prognosis (chance of recovery) for most young patients with nasopharyngeal cancer is very good.

Treatment

Treatment of nasopharyngeal cancer in children may include the following:

Young patients are more likely than adults to have problems caused by treatment, including second cancers.

See the PDQ summary on adult Nasopharyngeal Cancer Treatment for more information.

Esthesioneuroblastoma

Esthesioneuroblastoma (olfactoryneuroblastoma) is a tumor that begins in the olfactory bulb in the brain. The olfactory bulb connects to the nerve that is important to the sense of smell. Even though it is very rare, esthesioneuroblastoma is the most common tumor of the nasalcavity in children.

Most children have a tumor in the nose or nasal sinus at the time of diagnosis. The tumor may spread into the bone around the eyes, sinuses, and the front part of the brain. The disease rarely spreads to other parts of the body. Esthesioneuroblastoma usually appears during the teen years.

Signs and Symptoms and Staging Tests

Esthesioneuroblastoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Headache.
  • Blocked nose.
  • Nosebleeds.
  • Change in or loss of the sense of smell.
  • Bulging of the eye.
  • Frequent sinus infections.

Other conditions that are not esthesioneuroblastoma may cause these same signs and symptoms.

Esthesioneuroblastoma has usually spread by the time it is diagnosed. Tests to stage esthesioneuroblastoma may include the following:

See the General Information section for a description of these tests and procedures.

Prognosis

The prognosis (chance of recovery) depends on the following:

  • Whether the tumor was completely removed by surgery.
  • Whether the cancer is only in the nose or if it has spread to nearby nasal sinuses, lymph nodes, or to other parts of the body.

Treatment

Treatment of esthesioneuroblastoma in children may include the following:

  • Surgery for tumors that are in the nose only. External radiation therapy may also be given if the tumor was not completely removed.
  • Surgery followed by radiation therapy for tumors that have spread to the nasal sinus.
  • Chemotherapy and radiation therapy given alone or at the same time followed by surgery to remove the tumor for tumors that have spread outside the nose and nasal sinus but not to other parts of the body.
  • Chemotherapy, radiation therapy, and surgery for tumors that have spread to other parts of the body.

Thyroid Tumors

Thyroid tumors form in the tissues of the thyroid gland. The thyroid gland is a butterfly-shaped gland at the base of the throat near the windpipe. The thyroid gland makes important hormones that help control growth, heart rate, body temperature, and how quickly food is changed into energy.

The number of new cases of thyroid cancer in children, adolescents, and young adults has increased in recent years. Childhood thyroid tumors are more common in girls and children aged 15 to 19 years.

Thyroid tumors may be adenomas (noncancer) or carcinomas (cancer).

  • Adenoma: Adenomas can grow very large and sometimes make hormones. Adenomas may become malignant (cancer) and spread to the lungs or lymph nodes in the neck.
  • Carcinoma: There are three types of thyroid cancer:
    • Papillary. Papillary thyroid carcinoma is the most common type of thyroid cancer in children. It often spreads to the lymph nodes and may also spread to the lung. The prognosis (chance of recovery) for most patients is very good.
    • Follicular. Follicular thyroid carcinoma often spreads to the bone and lung. Sometimes it is inherited (passed from the parent to the child). The prognosis for most patients is very good.
    • Medullary. Medullary thyroid carcinoma is often inherited. It may have spread to other parts of the body at the time of diagnosis. The prognosis depends on the size of the tumor at the time of diagnosis.

Papillary and follicular thyroid carcinoma are often referred to as differentiated thyroid carcinoma.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of thyroid cancer is increased by the following:

Thyroid tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A lump in the neck.
  • A lump near the collarbone that does not hurt.
  • Trouble breathing.
  • Trouble swallowing.
  • Hoarseness or a change in the voice.
  • Hyperthyroidism (irregular heartbeat, shakiness, weight loss, trouble sleeping, frequent bowel movements, and sweating).

Other conditions that are not thyroid tumors may cause these same signs and symptoms.

Sometimes thyroid tumors do not cause any signs or symptoms.

Tests to diagnose and stage thyroid tumors may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose and stage thyroid tumors include the following:

  • Ultrasound : A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. This procedure can show the size of a thyroid tumor and whether it is solid or a fluid-filled cyst. Ultrasound may be used to guide a fine-needle aspiration (FNA) biopsy. A complete ultrasound exam of the neck is done before surgery.
  • Thyroid function test: The blood is checked for abnormal levels of thyroid-stimulating hormone (TSH). TSH is made by the pituitary gland in the brain. It stimulates the release of thyroid hormone and controls how fast follicular thyroid cells grow. The blood may also be checked for high levels of the hormone calcitonin.
  • Thyroid scan: If the amount of thyroid stimulating hormone in the child's blood is low, a scan to make images of the thyroid may be done before surgery. A small amount of a radioactive substance is swallowed or injected. The radioactive material collects in the thyroid gland. A special camera linked to a computer detects the radiation given off and makes pictures that show how the thyroid looks and functions.
  • Thyroglobulin test: The blood is checked for the amount of thyroglobulin, a protein made by the thyroid gland. Thyroglobulin levels are low or absent with normal thyroid function but may be higher with thyroid cancer or other conditions.

Prognosis

The prognosis (chance of recovery) depends on the following:

  • The child's gender.
  • The size of the tumor.
  • Whether the tumor has spread to lymph nodes or other parts of the body at the time of diagnosis.

Treatment

Treatment of papillary and follicular thyroid carcinoma in children may include the following:

  • Surgery to remove the thyroid gland and lymph nodes with cancer, followed by radioactive iodine (RAI) to kill any thyroid cancer cells that are left. Hormone replacement therapy (HRT) is given to make up for the lost thyroid hormone.
  • Radioactive iodine (RAI) for cancer that has recurred (come back).

Within 12 weeks of surgery, tests are done to find out if thyroid cancer remains in the body. These may include thyroglobulin tests and RAI scans. A radioactive iodine scan (RAI scan) is done to find areas in the body where thyroid cancer cells that were not removed during surgery may be dividing quickly. RAI is used because only thyroid cells take up iodine. A very small amount of RAI is swallowed, travels through the blood, and collects in thyroid tissue and thyroid cancer cells anywhere in the body. Further treatment depends on whether cancer cells remain in the body:

  • If no cancer cells are found outside the thyroid, a larger dose of RAI is given to destroy any remaining thyroid tissue.
  • If cancer remains in the lymph nodes or has spread to other parts of the body, an even larger dose of RAI is given to destroy any remaining thyroid tissue and thyroid cancer cells.

A whole-body SPECT (single photon emission computed tomography) scan may be done 4 to 7 days after treatment with RAI, to see if there are areas with cancer cells. A SPECT scan uses a special camera linked to a computer to make 3-dimensional (3-D) pictures of areas inside the body. A very small amount of a radioactive substance is injected into a vein. As the substance travels through the blood, the camera rotates around the body and takes pictures. Areas where thyroid cancer cells are growing will show up brighter in the picture. This procedure may be done just before or after a CT scan.

It is common for thyroid cancer to recur (come back), especially in children younger than 10 years and those with cancer in the lymph nodes. Ultrasound and thyroglobulin tests may be done from time to time to check if the cancer has recurred. Lifelong follow-up of thyroid hormone levels in the blood is needed to make sure the right amount of hormone replacement therapy (HRT) is being given. Talk with your child's doctor to find out how often these tests need to be done.

See the PDQ summary on adult Thyroid Cancer Treatment for more information.

Treatment of medullary thyroid carcinoma in children may include the following:

See the Multiple Endocrine Neoplasia Syndromes and Carney Complex section of this summary for more information.

Oral Cavity Cancer

Oral cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the mouth.

The oral cavity includes the following:

Most tumors in the oral cavity are benign (not cancer). The most common type of oral cavity cancer in adults, squamous cell carcinoma (cancer of the thin, flat cells lining the mouth), is very rare in children. Malignant tumors in children include lymphomas and sarcomas.

Signs and Symptoms, and Diagnostic and Staging Tests

Oral cavity cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A sore in the mouth that does not heal.
  • A lump or thickening in the oral cavity.
  • A white or red patch on the gums, tongue, or lining of the mouth.
  • Bleeding, pain, or numbness in the mouth.

Other conditions that are not oral cavity cancer may cause these same signs and symptoms.

Tests to diagnose and stage oral cavity cancer may include the following:

See the General Information section for a description of these tests and procedures.

Treatment

Treatment of oral cavity cancer in children may include the following:

Salivary Gland Tumors

Salivary gland tumors form in the salivary glands, which are small organs in the mouth and throat that make saliva. Most salivary gland tumors form in the parotid glands (just in front of and below each ear) or in the salivary glands under the tongue or near the jaw.

In children, most salivary gland tumors are benign (noncancer). Some salivary gland tumors are malignant (cancer), especially in young children. Malignant tumors sometimes form after treatment with radiation therapy and chemotherapy for leukemia or solid tumors.

Signs and Symptoms

Salivary gland tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A lump (usually painless) near the ear, cheek, jaw, or lip, or inside the mouth.
  • Fluid draining from the ear.
  • Trouble swallowing or opening the mouth widely.
  • Numbness or weakness in the face.
  • Pain in the face that does not go away.

Other conditions that are not salivary gland tumors may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stagesalivary gland cancer may include the following:

See the General Information section for a description of these tests and procedures.

Prognosis

The prognosis for salivary gland cancer is usually good.

Treatment

Treatment of salivary gland cancer includes the following:

See the PDQ summary on adult Salivary Gland Cancer Treatment for more information.

Laryngeal Cancer and Papillomatosis

Laryngeal Cancer

Laryngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the larynx. The larynx is also called the voice box. It's the part of the throat that has the vocal cords and is used in breathing, swallowing, and talking. Rhabdomyosarcoma (a malignant tumor of muscle) is the most common type of laryngeal cancer in children. Squamous cell carcinoma is a less common type of laryngeal cancer in children.

Signs and Symptoms for Laryngeal Cancer

Laryngeal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Hoarseness or a change in the voice.
  • Trouble or pain when swallowing.
  • A lump in the neck or throat.
  • A sore throat or cough that does not go away.
  • Ear pain.

Other conditions that are not laryngeal cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests for Laryngeal Cancer

Tests to diagnose and stage laryngeal cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose laryngeal cancer include the following:

  • Laryngoscopy : A procedure to look at the larynx (voice box) for abnormal areas. A mirror or a laryngoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth to see the larynx. A special tool on the laryngoscope may be used to remove samples of tissue. The tissues samples are viewed under a microscope by a pathologist to check for signs of cancer.
  • Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metalliccompound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.

Treatment of Laryngeal Cancer

Treatment of laryngeal cancer in children may include the following:

  • Chemotherapy and radiation therapy for rhabdomyosarcomas.
  • Laser surgery and radiation therapy for squamous cell cancer. Laser surgery uses a laser beam (a narrow beam of intense light) to turn the cancer cells into a gas that evaporates (dissolves into the air).

See the following PDQ summaries for more information:

Papillomatosis

Papillomatosis of the larynx is a condition that causes papillomas (benign tumors that look like warts) to form in the tissue that lines the larynx. Papillomatosis may be caused by the human papillomavirus (HPV). Papillomas in the larynx may block the airway and cause trouble breathing. These growths often recur (come back) after treatment and may become cancer of the larynx.

Treatment of Papillomatosis

Treatment of papillomatosis in children may include the following:

For papillomas that come back after being removed by laser surgery four times in one year, treatment may include:

Midline Tract Cancer with NUT Gene Changes (NUT Midline Carcinoma)

Midline tract cancer is a disease in which malignant (cancer) cells form in the respiratory tract and sometimes other places along the middle of the body. The respiratory tract is made up of the nose, throat, larynx, trachea, bronchi, and lungs. Cancer may also form in other places along the middle of the body, such as the thymus, the area between the lungs, the pancreas, liver, and bladder.

Midline tract cancer is caused by a change in a chromosome. Every cell in the body contains DNA (genetic material stored inside chromosomes) that controls how the cell looks and acts. Midline tract cancer may form when part of the DNA from chromosome 15 (called the NUTgene) moves to another chromosome, or when chromosome 15 is broken.

Prognosis

Midline tract cancer with NUT gene changes usually cannot be cured.

Treatment

There is no standard treatment for midline tract cancer with NUT gene changes. Treatment may include the following:


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Unusual Cancers of the Chest

Breast Cancer

Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. Breast cancer may occur in both male and female children.

Breast cancer is the most common cancer among females aged 15 to 39 years. Breast cancer in this age group is more aggressive and more difficult to treat than in older women. Treatments for younger and older women are similar. Younger patients with breast cancer may have genetic counseling (a discussion with a trained professional about inherited diseases) and testing for familial cancersyndromes. Also, the possible effects of treatment on fertility should be considered.

Most breast tumors in children are fibroadenomas, which are benign (not cancer). Rarely, these tumors become large phyllodes tumors (cancer) and begin to grow quickly. If a benign tumor begins to grow quickly, a fine-needle aspiration (FNA) biopsy or an excisional biopsy will be done. The tissues removed during the biopsy will be viewed under a microscope by a pathologist to check for signs of cancer.

Risk Factors, Signs, and Diagnostic and Staging Tests

The risk of breast cancer is increased by the following:

Breast cancer may cause any of the following signs. Check with your child’s doctor if your child has any of the following:

  • A lump or thickening in or near the breast or in the underarm area.
  • A change in the size or shape of the breast.
  • A dimple or puckering in the skin of the breast.
  • A nipple turned inward into the breast.
  • Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin that is around the nipple).
  • Dimples in the breast that look like the skin of an orange, called peau d’orange.

Other conditions that are not breast cancer may cause these same signs.

Tests to diagnose and stage breast cancer may include the following:

See the General Information section for a description of these tests and procedures.

Another test used to diagnose breast cancer is the mammogram (an x-ray of the breast). When treatment for another cancer included radiation therapy to the breast or chest, it is important to have a mammogram and MRI of the breast to check for breast cancer. These should be done beginning at age 25, or 10 years after finishing radiation therapy, whichever is later.

Treatment

Treatment of breast cancer in children may include the following:

  • Watchful waiting for benign tumors.
  • Surgery to remove the tumor, but not the whole breast. Radiation therapy may also be given.

See the PDQ summary Breast Cancer Treatment for more information on the treatment of adolescents and young adults with breast cancer.

Lung Cancer

Lung cancer begins in the tissue of the lung. The lungs are a pair of cone-shaped breathing organs in the chest. The lungs bring oxygen into the body as you breathe in. They release carbon dioxide, a waste product of the body’s cells, as you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung is slightly larger and has three lobes. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. Tiny air sacs called alveoli and small tubes called bronchioles make up the inside of the lungs.

In children, most lung tumors are malignant (cancer). The most common lung tumors are bronchial tumors and pleuropulmonary blastoma.

Bronchial Tumors

Bronchial tumors begin in the cells that line the surface of the lung. Most bronchial tumors in children are benign and occur in the trachea or large bronchi (large airways of the lung). Sometimes, a slow-growing bronchial tumor becomes cancer that may spread to other parts of the body.

Signs and Symptoms

Bronchial tumors may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

  • Coughing.
  • Wheezing.
  • Trouble breathing.
  • Spitting up blood from the airways or lung.
  • Frequent infections in the lung, such as pneumonia.

Other conditions that are not bronchial tumors may cause these same signs and symptoms. For example, symptoms of bronchial tumors are a lot like the symptoms of asthma, and that can make it hard to diagnose the tumor.

Diagnostic and Staging Tests

Tests to diagnose and stage bronchial tumors may include the following:

See the General Information section for a description of these tests and procedures.

A biopsy of the abnormal area is usually not done because it can cause severe bleeding.

Other tests used to diagnose bronchial tumors include the following:

  • Bronchography: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. A contrast dye is put through the bronchoscope to make the larynx, trachea, and airways show up more clearly on x-ray film.
  • Octreotide scan : A type of radionuclide scan used to find carcinoid and other types of tumors. A very small amount of radioactiveoctreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.

Prognosis

The prognosis (chance of recovery) for children with bronchial cancer is very good.

Treatment

Treatment of bronchial tumors in children may include the following:

See the Neuroendocrine Tumors (Carcinoid Tumors) section of this summary for more information.

Pleuropulmonary Blastoma

Pleuropulmonary blastomas (PPBs) form in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). PPBs can also form in the organs between the lungs including the heart, aorta, and pulmonaryartery, or in the diaphragm (the main breathing muscle below the lungs).

There are three types of PPB:

  • Type I tumors are cyst-like tumors in the lung. They are most common in children aged 2 years and younger and can usually be cured. Type Ir tumors are Type I tumors that have gotten smaller or have not grown or spread.
  • Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain.
  • Type III tumors are solid tumors. These tumors often spread to the brain.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of PPB is increased by the following:

PPB may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • A cough that doesn’t go away.
  • Trouble breathing.
  • Fever.
  • Lung infections, such as pneumonia.
  • Wheezing.
  • Pain in the chest or abdomen.
  • Loss of appetite.
  • Weight loss for no known reason.
  • Feeling very tired.

Other conditions that are not PPB may cause these same signs and symptoms.

Tests to diagnose and stage PPB may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose PPB include the following:

  • Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Thoracoscopy : A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If the thoracoscope cannot reach certain tissues, organs, or lymph nodes, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened.

PPBs may spread or recur (come back) even after being removed by surgery.

Prognosis

The prognosis (chance of recovery) depends on the following:

  • The type of pleuropulmonary blastoma.
  • Whether the tumor has spread to other parts of the body at the time of diagnosis.
  • Whether the tumor was completely removed by surgery.

Treatment

Treatment of pleuropulmonary blastoma in children includes the following:

  • Surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.

Esophageal Tumors

Esophageal tumors may be benign (not cancer) or malignant (cancer). Esophageal cancer is a disease in which malignant cells form in the tissues of the esophagus. The esophagus is the hollow, muscular tube that moves food and liquid from the throat to the stomach. Most esophageal tumors in children begin in the thin, flat cells that line the esophagus.

The esophagus and stomach are part of the upper gastrointestinal (digestive) system.

Signs and Symptoms

Esophageal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Trouble swallowing.
  • Streaks of blood in sputum (mucus coughed up from the lungs).
  • Weight loss.
  • Hoarseness and cough.
  • Indigestion and heartburn.
  • Vomiting with streaks of blood.

Other conditions that are not esophageal cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage esophageal cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose esophageal cancer include the following:

  • Esophagoscopy : A procedure to look inside the esophagus to check for abnormal areas. An esophagoscope is inserted through the mouth or nose and down the throat into the esophagus. An esophagoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A biopsy is usually done during an esophagoscopy. Sometimes a biopsy shows changes in the esophagus that are not cancer but may lead to cancer.
  • Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Thoracoscopy : A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. Sometimes this procedure is used to remove part of the esophagus or lung.
  • Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.

Prognosis

Esophageal cancer is hard to cure because it usually is not possible to remove the whole tumor by surgery.

Treatment

Treatment for esophageal cancer in children may include the following:

  • Radiation therapy given through a plastic or metal tube placed through the mouth into the esophagus.
  • Chemotherapy.
  • Surgery to remove all or part of the tumor.

See the PDQ summary on adult Esophageal Cancer for more information.

Thymoma and Thymic Carcinoma

Thymomas and thymic carcinomas are tumors of the thymus and the cells that cover the outside surface of the thymus. Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymoma and thymic carcinoma.

The thymus is a small organ in the upper chest under the breastbone. It is part of the lymph system and makes white blood cells, called lymphocytes, that help fight infection. Thymomas and thymic carcinomas usually form in the front part of the chest and are often found during a chest x-ray that is done for another reason.

Anatomy of the thymus gland. The thymus gland is a small organ that lies in the upper chest under the breastbone. It makes white blood cells, called lymphocytes, which protect the body against infections.

Thymoma and thymic carcinoma are slow-growing cancers. Thymic carcinomas are more likely than thymomas to spread to the lymph nodes or to other parts of the body.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

People who develop thymomas often have one of the following immune system diseases or hormonedisorders:

Thymoma and thymic carcinoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Coughing.
  • Trouble swallowing.
  • Hoarseness.
  • Pain or a tight feeling in the chest.
  • Trouble breathing.
  • Fever.
  • Weight loss.

Other conditions that are not thymoma or thymic carcinoma may cause these same signs and symptoms.

Tests to diagnose and stage thymoma and thymic carcinoma may include the following:

See the General Information section for a description of these tests and procedures.

Prognosis

The prognosis (chance of recovery) is better when the tumor has not spread. Most childhood thymomas are diagnosed before the tumor has spread.

Treatment

Treatment for thymoma and thymic carcinoma in children may include the following:

See the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.

Heart Tumors

Most tumors that form in the heart are benign (not cancer). Benign heart tumors that may appear in children include the following:

Before birth and in newborns, the most common benign heart tumors are teratomas. An inherited condition called tuberous sclerosis can cause heart tumors to form in a fetus or newborn.

Malignant tumors that begin in the heart are even more rare than benign heart tumors in children. Malignant heart tumors include:

Signs and Symptoms

Heart tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Change in the heart's normal rhythm.
  • Trouble breathing, especially when the child is lying down.
  • Pain in the middle of the chest that feels better when the child is sitting up.
  • Coughing.
  • Fainting.
  • Feeling dizzy, tired, or weak.
  • Fast heart rate.
  • Swelling in the legs, ankles, or abdomen.
  • Feeling anxious.
  • Signs of a stroke.
    • Sudden numbness or weakness of the face, arm, or leg (especially on one side of the body).
    • Sudden confusion or trouble speaking or understanding.
    • Sudden trouble seeing with one or both eyes.
    • Sudden trouble walking or feeling dizzy.
    • Sudden loss of balance or coordination.
    • Sudden severe headache for no known reason.

Sometimes heart tumors do not cause any signs or symptoms.

Other conditions that are not heart tumors may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage heart tumors may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose or stage heart tumors include the following:

  • Echocardiogram : A procedure in which high-energy sound waves (ultrasound) are bounced off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
  • Electrocardiogram (EKG): A recording of the heart's electrical activity to check its rate and rhythm. A number of small pads (electrodes) are placed on the patient’s chest, arms, and legs, and are connected by wires to the EKG machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.

Treatment

Treatment for heart tumors in children may include the following:

Mesothelioma

Malignant mesothelioma is a disease in which malignant (cancer) cells are found in the pleura (the thin layer of tissue that lines the chest cavity and covers the lungs) or the peritoneum (the thin layer of tissue that lines the abdomen and covers most of the organs in the abdomen). The tumors often spread over the surface of organs without spreading into the organ. They may spread to lymph nodes nearby or in other parts of the body. Malignant mesothelioma may also form in the heart or testicles, but this is rare.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

Mesothelioma is sometimes a late effect of treatment for an earlier cancer, especially after treatment with radiation therapy. In adults, mesothelioma has been linked to being exposed to asbestos, which was once used as building insulation. There is no information about the risk of mesothelioma in children exposed to asbestos.

Mesothelioma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Trouble breathing.
  • Pain under the rib cage.
  • Weight loss for no known reason.

Other conditions that are not mesothelioma may cause these same signs and symptoms.

Tests to diagnose and stage mesothelioma may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose mesothelioma include the following:

  • Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Thoracoscopy : A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung.
  • Thoracotomy : An incision (cut) is made between two ribs to check inside the chest for signs of disease.
  • Cytologic exam: An exam of cells under a microscope (by a pathologist) to check for anything abnormal. For mesothelioma, fluid is taken from around the lungs or from the abdomen. A pathologist checks the cells in the fluid.

Prognosis

The prognosis (chance of recovery) is better when the tumor has not spread.

Treatment

Treatment for mesothelioma in children may include the following:

See the PDQ summary on adult Malignant Mesothelioma Treatment for more information.


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Unusual Cancers of the Abdomen

Cancer of the Adrenal Cortex

There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Cancer of the adrenal cortex is also called adrenocortical carcinoma.

Childhood cancer of the adrenal cortex occurs most commonly in patients younger than 6 years or in the teen years, and more often in females.

The adrenal cortex makes important hormones that do the following:

  • Balance the water and salt in the body.
  • Help keep blood pressure normal.
  • Help control the body's use of protein, fat, and carbohydrates.
  • Cause the body to have male or female characteristics.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of cancer of the adrenal cortex is increased by having a certain mutation (change) in a gene or any of the following syndromes:

A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make extra hormones). Most tumors of the adrenal cortex in children are functioning tumors. The extra hormones made by functioning tumors may cause certain signs or symptoms of disease and these depend on the type of hormone made by the tumor. For example, extra androgen hormone may cause both male and female children to develop masculine traits, such as body hair or a deep voice, grow faster, and have acne. Extra estrogen hormone may cause the growth of breasttissue in male children. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the signs and symptoms of cancer of the adrenal cortex.)

The tests and procedures used to diagnose and stage adrenocortical carcinoma depend on the patient's symptoms. They may include:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose cancer of the adrenal cortex include the following:

  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these substances in the urine may be a sign of disease in the adrenal cortex.
  • Low-dosedexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
  • High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may be checked for testosterone or estrogen. A higher than normal amount of these hormones may be a sign of adrenocortical carcinoma.
  • Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
  • Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.

Prognosis

The prognosis (chance of recovery) is good for patients who have small tumors that have been completely removed by surgery. For other patients, the prognosis depends on the following:

  • Child's age.
  • Size of the tumor.
  • Whether the child has developed masculine traits.
  • Whether the covering around the tumor broke open during surgery to remove the tumor.

These tumors can spread to the kidneys, lungs, bones, and brain.

Treatment

Treatment for cancer of the adrenal cortex in children may include the following:

See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.

Stomach (Gastric) Cancer

Stomach cancer is a disease in which malignant (cancer) cells form in the lining of the stomach. The stomach is a J-shaped organ in the upper abdomen. It is part of the digestive system, which processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) in foods that are eaten and helps pass waste material out of the body. Food moves from the throat to the stomach through a hollow, muscular tube called the esophagus. After leaving the stomach, partly-digested food passes into the small intestine and then into the large intestine.

The esophagus and stomach are part of the upper gastrointestinal (digestive) system.

The risk of stomach cancer is increased by having an infection with Helicobacter pylori (H. pylori)bacterium, which is found in the stomach.

Signs and Symptoms

Many patients will have anemia (a lower than normal number of red blood cells), but have no signs or symptoms before the cancer spreads. Stomach cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Other conditions that are not stomach cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage stomach cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose stomach cancer include the following:

  • Upper endoscopy : A procedure to look inside the esophagus, stomach, and duodenum (first part of the small intestine) to check for abnormal areas. An endoscope is passed through the mouth and down the throat into the esophagus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.
  • Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metalliccompound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

Prognosis

Prognosis (chance of recovery) depends on whether the cancer has spread at the time of diagnosis.

Treatment

Treatment of stomach cancer in children may include the following:

See the PDQ summary on adult Gastric Cancer Treatment for more information.

Pancreatic Cancer

Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. The pancreas is a pear-shaped gland about 6 inches long. The wide end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. Many different kinds of tumors can form in the pancreas. Some tumors are benign (not cancer).

Anatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.

The pancreas has two main jobs in the body:

  • To make juices that help digest (break down) food. These juices are secreted into the small intestine.
  • To make hormones that help control the sugar and salt levels in the blood. These hormones are secreted into the bloodstream.

There are four types of pancreatic cancer in children:

  • Solid pseudopapillary tumor of the pancreas. This is the most common type of pancreatic tumor. It most commonly affects females that are older adolescents and young adults. The tumors have both cyst-like and solid parts. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is very good.
  • Pancreatoblastoma. It usually occurs in children aged 10 years or younger. Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These tumors may make adrenocorticotropic hormone (ACTH) and antidiuretic hormone (ADH). Pancreatoblastoma may spread to the liver, lungs, and lymph nodes. The prognosis for children with pancreatoblastoma is good.
  • Islet cell tumors. These tumors are not common in children and can be benign or malignant. Islet cell tumors may occur in children with multiple endocrine neoplasia type 1 (MEN1) syndrome. The most common types of islet cell tumors are insulinomas and gastrinomas. These tumors may make hormones, such as insulin and gastrin, that cause signs and symptoms.
  • Pancreatic carcinoma. Pancreatic carcinoma is very rare in children. The two types of pancreatic carcinoma are acinar cell carcinoma and ductal adenocarcinoma.

Signs and Symptoms

In children, some pancreatic tumors do not secrete hormones and there are no signs and symptoms of disease. This makes it hard to diagnose pancreatic cancer early.

Pancreatic tumors that do secrete hormones may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made.

If the tumor secretes insulin, signs and symptoms that may occur include the following:

  • Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
  • Changes in behavior.
  • Seizures.
  • Coma.

If the tumor secretes gastrin, signs and symptoms that may occur include the following:

Signs and symptoms caused by tumors that make other types of hormones may include the following:

  • Watery diarrhea.
  • Dehydration (feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired).
  • Low sodium (salt) level in the blood (confusion, sleepiness, muscle weakness, and seizures).
  • Weight loss or gain for no known reason.
  • Round face and thin arms and legs.
  • Feeling very tired and weak.
  • High blood pressure.
  • Purple or pink stretch marks on the skin.

If cancer is in the head of the pancreas, the bile duct or blood flow to the stomach may be blocked and the following signs may occur:

Check with your child’s doctor if you see any of these problems in your child. Other conditions that are not pancreatic cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage pancreatic cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose pancreatic cancer include the following:

  • Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
  • Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube, called a stent, may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs for cancer.
  • Somatostatin receptor scintigraphy : A type of radionuclide scan used to find pancreatic tumors. A very small amount of radioactiveoctreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is used to diagnose islet cell tumors.
  • Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.
  • Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.

Treatment

Treatment of solid pseudopapillary tumor of the pancreas in children may include the following:

  • Surgery to remove the tumor.
  • Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body.

Treatment of pancreatoblastoma in children may include the following:

  • Surgery to remove the tumor. A Whipple procedure may be done for tumors in the head of the pancreas.
  • Chemotherapy may be given to shrink the tumor before surgery. More chemotherapy may be given after surgery for large tumors, tumors that cannot be removed by surgery, and tumors that have spread to other parts of the body.
  • Chemotherapy may be given if the tumor does not respond to treatment or comes back.

Treatment of islet cell tumors in children may include drugs to treat symptoms caused by hormones and the following:

  • Surgery to remove the tumor.
  • Chemotherapy and targeted therapy for tumors that cannot be removed by surgery or that have spread to other parts of the body.

See the PDQ summaries on adult Pancreatic Cancer Treatment and adult Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment for more information on pancreatic tumors.

Colorectal Cancer

Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. The colon is part of the body’s digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6-8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body).

Anatomy of the lower digestive system, showing the colon and other organs.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

Childhood colorectal cancer is often part of an inheritedsyndrome that causes the disease. Some colorectal cancers in young people are linked to a genemutation that causes polyps (growths in the mucous membrane that lines the colon) to form that may turn into cancer later.

The risk of colorectal cancer is increased by having certain inherited conditions, such as:

Colon polyps that form in children who do not have an inherited syndrome are not linked to an increased risk of cancer.

Signs and symptoms of childhood colorectal cancer usually depend on where the tumor forms. Colorectal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Tumors of the rectum or lower colon may cause pain in the abdomen, constipation, or diarrhea.
  • Tumors in the part of the colon on the left side of the body may cause:

Other conditions that are not colorectal cancer may cause these same signs and symptoms.

Tests to diagnose and stage colorectal cancer may include the following:

Other tests used to diagnose colorectal cancer include the following:

  • Colonoscopy : A procedure to look inside the rectum and colon for polyps, abnormal areas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.
  • Barium enema : A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metalliccompound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series.
  • Fecal occult blood test : A test to check stool (solid waste) for blood that can only be seen with a microscope. Small samples of stool are placed on special cards and returned to the doctor or laboratory for testing.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • Kidney function test : A test in which blood or urine samples are checked for the amounts of certain substances released by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working the way they should. This is also called a renal function test.
  • Liver function test : A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.
  • Carcinoembryonic antigen (CEA) assay : A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of colorectal cancer or other conditions.

Prognosis

The prognosis (chance of recovery) depends on the following:

  • Whether the entire tumor was removed by surgery.
  • Whether the cancer has spread to other parts of the body, such as the lymph nodes, liver, pelvis, or ovaries.

Treatment

Treatment for colorectal cancer in children may include the following:

Children with certain familial colorectal cancer syndromes may be treated with:

  • Surgery to remove the colon before cancer forms.
  • Medicine to decrease the number of polyps in the colon.

See the following PDQ summaries on adult cancer for more information:

Neuroendocrine Tumors (Carcinoid Tumors)

Neuroendocrine tumors (including carcinoid tumors) usually form in the lining of the stomach or intestines, but they can form in other organs, such as the pancreas, lungs, or liver. These tumors are usually small, slow-growing, and benign (not cancer). Some neuroendocrine tumors are malignant (cancer) and spread to other places in the body. Sometimes neuroendocrine tumors in children form in the appendix (a pouch that sticks out from the first part of the large intestine near the end of the small intestine). The tumor is often found during surgery to remove the appendix.

See the Bronchial tumors section of this summary for information on bronchial carcinoid tumors.

Signs and Symptoms

Some neuroendocrine tumors release hormones and other substances. If the tumor is in the liver, high amounts of these hormones may remain in the body and cause a group of signs and symptoms called carcinoid syndrome. Carcinoid syndrome caused by the hormone somatostatin may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Redness and a warm feeling in the face and neck.
  • A fast heartbeat.
  • Trouble breathing.
  • Sudden drop in blood pressure (restlessness, confusion, weakness, dizziness, and pale, cool, and clammy skin).
  • Diarrhea.

Other conditions that are not neuroendocrine tumors may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests that check for signs of cancer are used to diagnose and stage neuroendocrine tumors. They may include:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose neuroendocrine tumors include the following:

  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The urine sample is checked to see if it contains a hormone made by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.
  • Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find tumors. A very small amount of radioactiveoctreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Prognosis

The prognosis for neuroendocrine tumors in the appendix in children is usually excellent after surgery to remove the tumor. Neuroendocrine tumors that are not in the appendix are usually larger or have spread to other parts of the body at the time of diagnosis and do not respond well to chemotherapy.

Treatment

Treatment for neuroendocrine tumors in the appendix in children may include the following:

  • Surgery to remove the appendix, when the tumor is small and only in the appendix.
  • Surgery to remove the appendix, lymph nodes, and part of the large intestine, when the tumor is larger, has spread to nearby lymph nodes, and is in the appendix.

Treatment for neuroendocrine tumors that have spread to the large intestine, pancreas, or stomach is the same as treatment for adult high-grade neuroendocrine tumors.

See the PDQ summary on adult Gastrointestinal Carcinoid Tumors Treatment for more information.

Gastrointestinal Stromal Tumors

Gastrointestinal stromal cell tumors (GIST) usually begin in cells in the wall of the stomach or intestines. GISTs may be benign (not cancer) or malignant (cancer). Childhood GISTs are more common in girls, and usually appear in the teen years.

Risk Factors and Signs and Symptoms

GISTs in children are not the same as GISTs in adults. Patients should be seen at centers that specialize in the treatment of GISTs and the tumors should be tested for genetic changes. A small number of children have tumors with genetic changes like those found in adult patients. The risk of GIST is increased by the following genetic disorders:

Most children with GIST have tumors in the stomach and develop anemia caused by bleeding. Signs and symptoms of anemia include the following:

  • Feeling tired.
  • Dizziness.
  • A fast or irregular heartbeat.
  • Shortness of breath.
  • Pale skin.

A lump in the abdomen or a blockage of the intestine (crampy pain in the abdomen, nausea, vomiting, diarrhea, constipation, and swelling of the abdomen) are also signs of GIST.

Other conditions that are not anemia caused by GIST may cause these same signs and symptoms.

Treatment

Treatment for children who have tumors with genetic changes like those found in adult patients is targeted therapy with a tyrosine kinase inhibitor.

Treatment for children whose tumors do not show genetic changes may include the following:

  • Surgery to remove the tumor and check nearby lymph nodes for signs of cancer. If cancer is in the lymph nodes, the lymph nodes are removed.
  • Watchful waiting for tumors that come back in the same place or cannot be removed, but do not cause signs or symptoms.
  • Targeted therapy with a tyrosine kinase inhibitor.

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Unusual Cancers of the Reproductive and Urinary Systems

Bladder Cancer

Bladder cancer is a disease in which malignant (cancer) cells form in the tissues of the bladder. The bladder is a hollow organ in the lower part of the abdomen. It is shaped like a small balloon and has a muscle wall that allows it to get bigger or smaller. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body.

Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.

The most common type of bladder cancer is transitional cell cancer. Squamous cell and other more aggressive types of bladder cancer are less common.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of bladder cancer is increased in females who have been treated for cancer with certain anticancer drugs called alkylating agents.

Bladder cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Blood in the urine (slightly rusty to bright red in color).
  • Frequent urination or feeling the need to urinate without being able to do so.
  • Pain during urination.
  • Lower back pain.

Other conditions that are not bladder cancer may cause the same signs and symptoms.

Tests to diagnose and stage bladder cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose bladder cancer include the following:

Prognosis

In children, bladder cancer is usually low grade (not likely to spread) and the prognosis is usually excellent after surgery to remove the tumor.

Treatment

Treatment for bladder cancer in children is usually transurethral resection (TUR). This is a surgical procedure to remove tissue from the bladder using a resectoscope inserted into the bladder through the urethra. A resectoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool to remove tissue and burn away any remaining tumor cells. Tissue samples are checked under a microscope for signs of cancer.

See the PDQ summary on adult Bladder Cancer Treatment for more information.

Testicular Cancer

Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles. The testicles are 2 egg-shaped glands located inside the scrotum (a sac of loose skin that lies directly below the penis). The testicles are held within the scrotum by the spermatic cord, which also contains the vas deferens and vessels and nerves of the testicles.

Anatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs.

There are two types of testicular tumors:

  • Germ cell tumors: Tumors that start in sperm cells in males. Testicular germ cell tumors may be benign (not cancer) or malignant (cancer). The most common testicular germ cell tumors in young boys are benign teratomas and malignant nonseminomas. Seminomas usually occur in young men and are rare in boys.
  • Non-germ cell tumors: Tumors that begin in the tissues that surround and support the testicles. These tumors may be benign or malignant.

Signs and Symptoms and Diagnostic and Staging Tests

Testicular cancer and its spread to other parts of the body may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

A painless lump in the testicles may be a sign of a testicular tumor. Other conditions may also cause a lump in the testicles.

Tests to diagnose and stage non-germ cell testicular cancer may include the following:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose testicular tumors include the following:

  • Serum tumor marker test : A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The tumor marker alpha-fetoprotein is used to diagnose germ cell tumors.

Treatment

Treatment for non-germ cell testicular cancer in children may be surgery to remove the tumor.

See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on testicular germ cell tumors.

Ovarian Cancer

Ovarian cancer is a disease in which malignant (cancer) cells form in the ovary. The ovaries are a pair of organs in the female reproductive system. They are located in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries produce eggs and female hormones (chemicals that control the way certain cells or organs function).

Anatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.

Most ovariantumors in children are benign (not cancer). They occur most often in females aged 15 to 19 years.

There are several types of malignant ovarian tumors:

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of ovarian cancer is increased by having one of the following conditions:

Ovarian cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Other conditions that are not ovarian cancer may cause these same signs and symptoms.

Tests to diagnose and stage ovarian cancer may include the following:

See the General Information section for a description of these tests and procedures.

During surgery to remove the tumor, fluid in the abdomen will be checked for signs of cancer.

Prognosis

Ovarian epithelial cancer is usually found at an early stage in children and is easier to treat than in adult patients.

Treatment

Treatment of ovarian epithelial cancer in children may include the following:

Treatment of ovarian stromal tumors in children may include the following:

  • Surgery to remove one ovary and one fallopian tube for early cancer.
  • Surgery followed by chemotherapy for cancer that is advanced.
  • Chemotherapy for cancer that has recurred (come back).

See the following PDQ summaries for more information:

Cervical and Vaginal Cancer

Cervical cancer is a disease in which malignant (cancer) cells form in the cervix. The cervix is the lower, narrow end of the uterus (the hollow, pear-shaped organ where a fetus grows). The cervix leads from the uterus to the vagina (birth canal). Vaginal cancer forms in the vagina. The vagina is the canal leading from the cervix to the outside of the body. At birth, a baby passes out of the body through the vagina (also called the birth canal).

Anatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.

The most common sign of cervical and vaginal cancer is bleeding from the vagina. Other conditions may also cause vaginal bleeding. Children are often diagnosed with advanced disease.

Treatment

Treatment for childhood cervical and vaginal cancer may include surgery to remove as much of the cancer as possible, followed by radiation therapy. Chemotherapy may also be used but it is not yet known how well this treatment works.


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Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes

Multiple endocrine neoplasia (MEN) syndromes are inheriteddisorders that affect the endocrine system. The endocrine system is made up of glands and cells that make hormones and release them into the blood. MEN syndromes may cause hyperplasia (the growth of too many normal cells) or tumors that may be benign (not cancer) or malignant (cancer).

There are several types of MEN syndromes and each type may cause different conditions or cancers. Patients and family members with an increased risk of these syndromes should have genetic counseling and tests to check for the syndromes.

The two main types of MEN syndromes are MEN1 and MEN2:

MEN1 syndrome is also called Werner syndrome. This syndrome usually causes tumors in the parathyroid gland, pituitary gland, and pancreas. Rarely, it causes tumors in the adrenal glands, gastrointestinal tract, fibroustissue, and fat cells. The tumors make extra hormones and cause certain signs or symptoms of disease. The signs and symptoms depend on the type of hormone made by the tumor.

The most common sign of MEN1 syndrome is hypercalcemia. Hypercalcemia occurs when the parathyroid gland makes too much parathyroid hormone. Signs and symptoms of hypercalcemia include the following:

A diagnosis of MEN1 syndrome is usually made when tumors are found in two different places. The prognosis (chance of recovery) is usually good.

Children who are diagnosed with MEN1 syndrome are checked for signs of cancer starting at age 5 and continuing for the rest of their life. Talk to your doctor about the tests and procedures that should be done to check for signs of cancer and how often they should be done.

Children with MEN1 syndrome may also have primary hyperparathyroidism. In primary hyperparathyroidism, one or more of the parathyroid glands makes too much parathyroid hormone. The most common sign of primary hyperparathyroidism is kidney stones. Children with primary hyperparathyroidism may have genetic testing to check for gene changes linked to MEN1 syndrome.


MEN2 syndrome includes three subgroups:
  • MEN2A syndrome

    MEN2A syndrome is also called Sipple syndrome. A diagnosis of MEN2A syndrome may be made when the patient or the patient's parents, brothers, sisters, or children have two or more of the following:

    Signs and symptoms of medullary thyroid cancer may include:

    • A lump in the throat or neck.
    • Trouble breathing.
    • Trouble swallowing.
    • Hoarseness.

    Signs and symptoms of pheochromocytoma may include:

    • Pain in the abdomen or chest.
    • A strong, fast, or irregular heartbeat.
    • Headache.
    • Heavy sweating for no known reason.
    • Dizziness.
    • Feeling shaky.
    • Being irritable or nervous.

    Signs and symptoms of parathyroid disease may include:

    • Hypercalcemia.
    • Pain in the abdomen, side, or back that doesn't go away.
    • Pain in the bones.
    • A broken bone.
    • A lump in the neck.
    • Change in voice, such as hoarseness.
    • Trouble swallowing.

    Family members of patients with the MEN2A syndrome should have genetic counseling and be tested in early childhood, before age 5, for the gene changes that lead to this type of cancer.

    A small number of medullary thyroid cancers may occur at the same time as Hirschsprung disease (chronic constipation that begins when a child is an infant), which has been found in some families with MEN2A syndrome. Hirschsprung disease may appear before other signs of MEN2A syndrome do. Patients who are diagnosed with Hirschsprung disease should be checked for certain gene changes that cause MEN2A syndrome.

    Familial medullary carcinoma of the thyroid (FMTC) is a type of MEN2A syndrome that causes medullary thyroid cancer. A diagnosis of FMTC may be made when two or more family members have medullary thyroid cancer and no family members have parathyroid or adrenal gland problems.

  • MEN2B syndrome

    Patients with MEN2B syndrome may have a slender body build with long, thin arms and legs. The lips may appear thick and bumpy because of benign tumors in the mucous membranes. MEN2B syndrome may cause the following conditions:

    • Medullary thyroid cancer.
    • Parathyroid hyperplasia.
    • Adenomas.
    • Pheochromocytoma.
    • Nerve cell tumors in the mucous membranes or other places.

Tests used to diagnose and stage MEN syndromes depend on the signs and symptoms and the patient's family history. They may include:

See the General Information section for a description of these tests and procedures.

Other tests and procedures used to diagnose MEN syndromes include the following:

  • Genetic testing : A laboratory test that analyzes cells or tissues to look for changes in a gene, chromosome, or protein. These changes may be a sign of a genetic disease or condition. They may also be linked to an increased risk of developing a specific disease or condition.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may be checked for abnormal levels of thyroid-stimulating hormone (TSH). TSH is made by the pituitary gland in the brain. It stimulates the release of thyroid hormone and controls how fast follicular thyroid cells grow. The blood may also be checked for high levels of the hormone calcitonin or parathyroid hormone (PTH).
  • Radioactive iodine scan (RAI scan): A procedure to find areas in the body where thyroid cancer cells may be dividing quickly. Radioactive iodine (RAI) is used because only thyroid cells take up iodine. A very small amount of RAI is swallowed, travels through the blood, and collects in thyroid tissue and thyroid cancer cells anywhere in the body. Abnormal thyroid cells take up less iodine than normal thyroid cells do. Areas that do not take up the iodine normally are called cold spots. Cold spots show up lighter in the picture made by the scan. They can be either benign (not cancer) or malignant, so a biopsy is done to find out if they are cancer.
  • Sestamibi scan : A type of radionuclide scan used to find an overactive parathyroid gland. A very small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity.
  • Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into a blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.
  • Venous sampling for an overactive parathyroid gland: A procedure in which a sample of blood is taken from veins near the parathyroid glands. The sample is checked to measure the amount of parathyroid hormone released into the blood by each gland. Venous sampling may be done if blood tests show there is an overactive parathyroid gland but imaging tests don’t show which one it is.
  • Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
  • MIBG scan : A procedure used to find neuroendocrine tumors, such as pheochromocytoma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma.
  • Pentagastrin stimulation test: A test in which blood samples are checked to measure the amount of calcitonin in the blood. Calcium gluconate and pentagastrin are injected into the blood and then several blood samples are taken over the next 5 minutes. If the level of calcitonin in the blood increases, it may be a sign of medullary thyroid cancer.

Treatment

There are several types of MEN syndrome, and each type may need different treatment:

  • Patients with the MEN1 syndrome are treated for parathyroid, pancreatic and pituitary tumors.
  • Patients with the MEN1 syndrome and primary hyperparathyroidism may have surgery to remove at least three parathyroid glands and the thymus.
  • Patients with the MEN2A syndrome usually have surgery to remove the thyroid by age 5 or earlier if genetic tests show certain changes in the RET gene. The surgery is done to diagnose cancer or to prevent cancer from forming or spreading.
  • Infants with the MEN2B syndrome may have surgery to remove the thyroid to prevent cancer.
  • Children with the MEN2B syndrome who have medullary thyroid cancer may be treated with targeted therapy (kinase inhibitor).
  • Patients with Hirschsprung disease and certain gene changes may have the thyroid removed to prevent cancer.

Pheochromocytoma and Paraganglioma

Pheochromocytoma and paraganglioma are rare tumors that come from the same type of nervetissue.

  • Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Pheochromocytoma is a tumor of the adrenal medulla. The adrenal glands make important hormones called catecholamines. Adrenaline (epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Some pheochromocytomas release extra adrenaline and noradrenaline into the blood and cause symptoms.
  • Paraganglioma forms outside the adrenal glands near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of extra adrenaline and noradrenaline into the blood may cause symptoms.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor doesn't mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.

The risk of pheochromocytoma or paraganglioma is increased by having any of the following inheritedsyndromes or gene changes:

More than half of the children and adolescents diagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increased the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.

Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test or procedure is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other symptoms may be caused by pheochromocytoma, paraganglioma, or other conditions. Check with your child’s doctor if your child has any of the following:

  • High blood pressure.
  • Headache.
  • Heavy sweating for no known reason.
  • A strong, fast, or irregular heartbeat.
  • Feeling shaky.
  • Being extremely pale.
  • Dizziness.
  • Being irritable or nervous.

These signs and symptoms may come and go but high blood pressure is more likely to occur for long periods of time in young patients. These signs and symptoms may also occur with physical activity, injury, anesthesia, surgery to remove the tumor, eating foods such as chocolate and cheese, or while passing urine (if the tumor is in the bladder).

Tests used to diagnose and stage pheochromocytoma and paraganglioma depend on the signs and symptoms and the patient's family history. They may include:

See the General Information section for a description of these tests and procedures.

Other tests and procedures used to diagnose pheochromocytoma and paraganglioma include the following:

  • Plasma-free metanephrines test: A blood test that measures the amount of metanephrines in the blood. Metanephrines are substances that are made when the body breaks down adrenaline or noradrenaline. Pheochromocytomas and paragangliomas can make large amounts of adrenaline and noradrenaline and cause high levels of metanephrines in both the blood and urine.
  • Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines (adrenaline or noradrenaline) released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma or paraganglioma.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines (adrenaline or noradrenaline) or metanephrines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma or paraganglioma.
  • MIBG scan : A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Treatment

Treatment of pheochromocytoma and paraganglioma in children may include the following:

Before surgery, drug therapy with alpha-blockers to control blood pressure and beta-blockers to control heart rate are given. If both adrenal glands are removed, life-long hormone therapy to replace hormones made by the adrenal glands is needed after surgery.

Skin Cancer (Melanoma, Squamous Cell Cancer, Basal Cell Cancer)

Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the body’s largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells:

  • Melanocytes: Found in the lower part of the epidermis, these cells make melanin, the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment and cause the skin to darken.
  • Squamous cells: Thin, flat cells that form the top layer of the epidermis.
  • Basal cells: Round cells under the squamous cells.
Anatomy of the skin, showing the epidermis, dermis, and subcutaneous tissue. Melanocytes are in the layer of basal cells at the deepest part of the epidermis.

There are three types of skin cancer:

Melanoma

Even though melanoma is rare, it is the most common skin cancer in children. It occurs more often in adolescents aged 15 to 19 years.

The risk of having melanoma is increased by having the following conditions:

Other risk factors for melanoma in all age groups include:

  • Having a fair complexion, which includes the following:
    • Fair skin that freckles and burns easily, does not tan, or tans poorly.
    • Blue or green or other light-colored eyes.
    • Red or blond hair.
  • Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.
  • Having several large or many small moles.
  • Having a family history or personal history of unusual moles (atypical nevus syndrome).
  • Having a family history of melanoma.

Signs and symptoms of melanoma include the following:

  • A mole that:
    • changes in size, shape, or color.
    • has irregular edges or borders.
    • is more than one color.
    • is asymmetrical (if the mole is divided in half, the 2 halves are different in size or shape).
    • itches.
    • oozes, bleeds, or is ulcerated (a hole forms in the skin when the top layer of cells breaks down and the tissue below shows through).
  • Change in pigmented (colored) skin.
  • Satellite moles (new moles that grow near an existing mole).

Tests to diagnose and stage melanoma may include the following:

See the General Information section for a description of these tests and procedures.

Other tests and procedures used to diagnose melanoma include the following:

  • Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.
  • Biopsy : All or part of the abnormal-looking growth is cut from the skin and viewed under a microscope by a pathologist to check for cancer cells. There are four main types of skin biopsies:
    • Shave biopsy: A sterile razor blade is used to “shave off” the abnormal-looking growth.
    • Punch biopsy: A special instrument called a punch or a trephine is used to remove a circle of tissue from the abnormal-looking growth.
    • Excisional biopsy: A scalpel is used to remove the entire growth.
    • Wide local excision: A scalpel is used to remove the growth and some of the normal tissue around the area, to check for cancer cells. Skin grafting may be needed to cover the area where tissue was removed.
  • Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes.
  • Lymph node dissection : A surgical procedure in which lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called a lymphadenectomy.

Treatment of Melanoma

Treatment for melanoma is surgery to remove the tumor and some tissue around the tumor.

If cancer has spread to nearby lymph nodes, treatment is surgery to remove the lymph nodes with cancer. Immunotherapy with high-doseinterferon may also be given.

Treatment for melanoma that has spread beyond the lymph nodes may include the following:

See the PDQ summary on adult Melanoma Treatment for more information.

Squamous Cell and Basal Cell Skin Cancer

The risk of squamous cell or basal cell cancer is increased by the following:

  • Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.
  • Having a fair complexion, which includes the following:
    • Fair skin that freckles and burns easily, does not tan, or tans poorly.
    • Blue or green or other light-colored eyes.
    • Red or blond hair.
  • Having actinic keratosis.
  • Past treatment with radiation.
  • Having a weakened immune system.

Signs of squamous cell and basal cell skin cancer include the following:

  • A sore that does not heal.
  • Areas of the skin that are:
    • Small, raised, smooth, shiny, and waxy.
    • Small, raised, and red or reddish-brown.
    • Flat, rough, red or brown, and scaly.
    • Scaly, bleeding, or crusty.
    • Similar to a scar and firm.

Tests to diagnose squamous cell and basal cell skin cancer include the following:

  • Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.
  • Biopsy: All or part of a growth that doesn't look normal is cut from the skin and viewed under a microscope by a pathologist to check for signs of cancer. There are three main types of skin biopsies:
    • Shave biopsy: A sterile razor blade is used to “shave off” the growth that does not look normal.
    • Punch biopsy: A special instrument called a punch or a trephine is used to remove a circle of tissue from the growth that does not look normal.
    • Excisional biopsy: A scalpel is used to remove the entire growth.

Treatment of Squamous Cell and Basal Cell Skin Cancer

Treatment of squamous cell and basal cell cancer in children may include the following:

  • Surgery to remove the tumor.

See the PDQ summary on adult Skin Cancer Treatment for more information.

Chordoma

Chordoma is a very rare type of bone tumor that forms anywhere along the spine from the base of the skull to the tailbone. In children and adolescents, chordomas develop more often in the base of the skull, making them hard to remove completely with surgery.

Childhood chordoma is linked to the conditiontuberous sclerosis, a geneticdisorder in which tumors that are benign (not cancer) form in the kidneys, brain, eyes, heart, lungs, and skin.

Signs and Symptoms

Chordoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

  • Headache.
  • Neck or back pain.
  • Double vision.
  • Paralysis of the muscles in the face.
  • Numbness, tingling, or weakness of the arms and legs.
  • A change in bowel or bladder habits.

Other conditions that are not chordoma may cause these same signs and symptoms.

Chordomas may recur (come back), usually in the same place, but sometimes they recur in other areas of bone or in the lungs.

Treatment

Treatment of chordoma in children may include the following:

Cancer of Unknown Primary Site

Carcinoma of unknown primary is a rare disease in which malignant (cancer) cells are found in the body but the place the cancer began is not known. Cancer can form in any tissue of the body. The primary cancer (the cancer that first formed) can spread to other parts of the body. This process is called metastasis. Cancer cells usually look like the cells in the type of tissue in which the cancer began. For example, breast cancer cells may spread to the lung. Because the cancer began in the breast, the cancer cells in the lung look like breast cancer cells.

Sometimes doctors find where the cancer has spread but cannot find where in the body the cancer first began to grow. This type of cancer is called a cancer of unknown primary or occult primary tumor.

In carcinoma of unknown primary, cancer cells have spread in the body but the place where the primary cancer began is unknown.

Tests are done to find where the primary cancer began and to get information about where the cancer has spread. When tests are able to find the primary cancer, the cancer is no longer a cancer of unknown primary and treatment is based on the type of primary cancer.

Because the place where the cancer started is not known, many different tests and procedures may be needed to find out what type of cancer it is. If tests show there may be cancer, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist. The pathologist views the tissue to look for cancer cells and to find out the type of cancer. The type of biopsy that is done depends on the part of the body being tested for cancer. One of the following types of biopsies may be used:

When the type of cancer cells or tissue removed is different from the type of cancer cells expected to be found, a diagnosis of cancer of unknown primary may be made. The cells in the body have a certain look that depends on the type of tissue they come from. For example, a sample of cancer tissue taken from the breast is expected to be made up of breast cells. However, if the sample of tissue is a different type of cell (not made up of breast cells), it is likely that the cells have spread to the breast from another part of the body.

Adenocarcinomas, melanomas, and embryonal tumors are common tumor types that appear and it is not known where the cancer first formed. Embryonal tumors such as rhabdomyosarcomas and neuroblastomas are most common in children.

Treatment

Treatment depends on what the cancer cells look like under a microscope, the patient's age, signs and symptoms, and where the cancer has spread in the body. Treatment is usually chemotherapy, targeted therapy, or radiation therapy.

See the PDQ summary on adult Carcinoma of Unknown Primary for more information.


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To Learn More About Childhood Cancer

For more information from the National Cancer Institute about unusual cancers of childhood, see the following:

For more childhood cancer information and other general cancer resources, see the following:


Back to Top Source: The National Cancer Institute's Physician Data Query (PDQ®) Cancer Information Summaries (http://www.cancer.gov/cancertopics/pdq)