National Cancer Institute


Posted Date: Apr 27, 2014

Expert-reviewed information summary about the treatment of unusual cancers of childhood such as cancers of the head and neck, chest, abdomen, reproductive system, skin, and others.

Unusual Cancers of Childhood

General Information About Unusual Cancers of Childhood

Key Points for this Section

  • Unusual cancers of childhood are cancers rarely seen in children.
  • Tests are used to detect (find), diagnose, and stage unusual cancers of childhood.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

Unusual cancers of childhood are cancers rarely seen in children.

Cancer in children and teenagers 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 might 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:

  • 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 in the organ or tissue that makes it.
  • 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:Excisional biopsy: The removal of an entire lump or area of tissue that doesn’t look normal.Incisional biopsy: The removal of part of a lump or a sample of tissue that doesn’t look normal. Core biopsy: The removal of tissue using a wide needle. Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
  • 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 patient lies on a table that slides through the CT machine, which takes x-ray pictures of the inside of the body.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (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.
  • 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 patient lies on a table that slides into the MRI machine, which takes pictures of the inside of the body. The pad on the patient’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 passed over the surface of the abdomen. The ultrasound 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 and is detected by a scanner.Bone scan. A small amount of radioactive material is injected into the patient's bloodstream and collects in abnormal cells in the bones. As the patient lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen or film.

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.

Treatment Option Overview

Key Points for this Section

  • There are different types of treatment for children with unusual cancers.
  • Children with unusual cancers should have their treatment planned by a team of health care providers with expertise in treating cancer in children.
  • Seven types of standard treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
    • Hormone therapy
    • Biologic therapy
    • Watchful waiting
    • Targeted therapy
  • New types of treatment are being tested in clinical trials.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.
  • Some cancers and cancer treatments cause side effects months or years after treatment has ended.

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 with expertise 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 pediatric health 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 two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance that is injected into the body or sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

Radiosurgery and proton beam therapy are two kinds of external radiation therapy used to treat childhood cancers:

  • Radiosurgery uses special equipment to aim one large dose of radiation directly at a tumor, causing less damage to nearby healthy tissue. It is also called stereotaxic radiosurgery, stereotactic radiosurgery, and radiation surgery. This procedure does not remove the tumor in an operation.
  • Proton beam radiation therapy is a type of high-energy radiation therapy that uses streams of protons (small, positively-charged particles of matter) to kill tumor cells.

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.

Biologic therapy

Biologic therapy 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 immunotherapy.

Interferon-beta is a type of biologic therapy used to treat nasopharyngeal cancer.

EBV-specific cytotoxic T-lymphocytes is another a type of biologic therapy used to treat nasopharyngeal cancer. White blood cells (T-lymphocytes) that are treated in the laboratory with Epstein-Barr virus are given to the patient to stimulate the immune system and fight cancer.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting may be a treatment option 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. Tyrosine kinase inhibitors (TKIs) are targeted therapy drugs that block signals needed for tumors to grow. Vascular endothelial growth factor (VEGF) inhibitors are another type of targeted therapy that prevents the growth of new blood vessels that tumors need to grow.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

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. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

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. This is sometimes called re-staging.

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).

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 nasal cavity (inside of the nose) and throat. It is rare in children younger than 10 and more common in teenagers.

Risk Factors, 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.

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

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

Other conditions that are not nasopharyngeal cancer may cause these same 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:

  • Nasoscopy: A procedure in which a doctor inserts a nasoscope (a thin, lighted tube) into the patient’s nose to look for abnormal areas.
  • 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.
  • 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. The prognosis and treatment options depend on the following:

  • The size of the tumor at diagnosis.
  • Whether the tumor has spread to nearby tissues, lymph nodes, or distant parts of the body.
  • How the cancer responds to the initial treatment.

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

Esthesio neuroblastoma ( olfactory neuroblastoma) 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 rare, esthesioneuroblastoma is the most common tumor of the nasal cavity in children.

Most children have a tumor in the nose or throat 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 is more common in boys and usually appears during the teen years.

Symptoms and Staging Tests

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

  • Blocked nose.
  • Nosebleeds.
  • Loss of the sense of smell.
  • Bulging of the eye.
  • Frequent sinus infections.

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

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

  • PET- CT scan: Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.

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

Prognosis

The prognosis (chance of recovery) depends on whether the cancer is only in the nose or if it has spread to nearby lymph nodes or to other parts of the body.

Treatment

Treatment of esthesioneuroblastoma in children may include the following:

Thyroid Tumors

Thyroid tumors form in the tissues of the thyroid gland, which 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.

Most childhood thyroid tumors occur 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. Thyroid cancer usually grows and spreads slowly.
  • Carcinoma: There are 3 types of thyroid cancer:Papillary.Follicular.Medullary.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of thyroid cancer is increased by being exposed to radiation and by certain genetic syndromes, such as multiple endocrine neoplasia (MEN) type 2A syndrome or multiple endocrine neoplasia (MEN) type 2B syndrome. See the Multiple Endocrine Neoplasia Syndromes and Carney Complex section of this summary for more information.

Thyroid tumors may cause any of the following symptoms. Check with your child’s doctor if you see any of the following problems in your child:

  • A lump in the neck or near the collarbone.
  • Trouble breathing.
  • Trouble swallowing.
  • Hoarseness or a change in the voice.

Other conditions that are not thyroid tumors may cause these same 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.
  • 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.
  • 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:

  • Gender.
  • The size of the tumor.
  • Whether the tumor has spread to other parts of the body at diagnosis.

Treatment

Treatment of thyroid tumors in children may include the following:

Four to six weeks after surgery 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. If no cancer cells are found, 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.

It is common for thyroid cancer to recur, especially in children younger than 10 years and those with cancer in the lymph nodes. 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. It is possible that thyroid cancer will spread to the lung later. Tests are done to check for thyroid cancer in the lung.

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

Oral Cavity Cancer

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

The number of new cases of cancer of the


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