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Unusual Cancers of Childhood Treatment (PDQ)


General Information About Unusual Cancers of Childhood
Treatment Option Overview
Unusual Cancers of the Head and Neck
Unusual Cancers of the Chest
Unusual Cancers of the Abdomen
Unusual Cancers of the Reproductive and Urinary Systems
Other Rare Unusual Cancers of Childhood
To Learn More About Childhood Cancer
About This PDQ Summary

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:

There are three ways that cancer spreads in the body.

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

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.

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

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 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 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.

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. Types of targeted therapies used to treat unusual childhood cancers include the following:

Targeted therapies are being studied in the treatment of other unusual cancers of childhood.

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 foreign genetic 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:

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 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:

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:

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 (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 very rare, esthesioneuroblastoma is the most common tumor of the nasal cavity 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:

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:

Treatment

Treatment of esthesioneuroblastoma in children may include the following:

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.

Anatomy of the thyroid and parathyroid glands; drawing shows the thyroid gland at the base of the throat near the trachea. An inset shows the front and back views. The front view shows that the thyroid is shaped like a butterfly, with the right lobe and left lobe connected by a thin piece of tissue called the isthmus. The back view shows the four pea-sized parathyroid glands. The larynx is also shown.Anatomy of the thyroid and parathyroid glands. The thyroid gland lies at the base of the throat near the trachea. It is shaped like a butterfly, with the right lobe and left lobe connected by a thin piece of tissue called the isthmus. The parathyroid glands are four pea-sized organs found in the neck near the thyroid. The thyroid and parathyroid glands make hormones.

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).

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:

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:

Prognosis

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

Treatment

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

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:

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:

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:

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

Diagnostic and Staging Tests

Tests to diagnose and stage salivary 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:

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:

Treatment of Laryngeal Cancer

Treatment of laryngeal cancer in children may include the following:

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 NUT gene) 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:

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 cancer syndromes. 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:

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:

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.

Respiratory anatomy; drawing shows right lung with upper, middle, and lower lobes; left lung with upper and lower lobes; and the trachea, bronchi, lymph nodes, and diaphragm. Inset shows bronchioles, alveoli, artery, and vein.  

Anatomy of the respiratory system, showing the trachea and both lungs and their lobes and airways. Lymph nodes and the diaphragm are also shown. Oxygen is inhaled into the lungs and passes through the thin membranes of the alveoli and into the bloodstream (see inset).

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:

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:

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 pulmonary artery, or in the diaphragm (the main breathing muscle below the lungs).

There are three types of PPB:

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:

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:

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

Prognosis

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

Treatment

Treatment of pleuropulmonary blastoma in children includes the following:

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.

Gastrointestinal (digestive) system anatomy; drawing shows the esophagus, liver, stomach, small intestine, and large intestine.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:

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:

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:

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

Thymoma

Thymoma is a rare tumor of the cells that cover the outside surface of the thymus. 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. Thymoma usually forms in the front part of the chest and is often found during a chest x-ray that is done for another reason.

Anatomy of the thymus gland; drawing shows the thymus gland in the upper chest under the breastbone. Also shown are the ribs, lungs, and heart.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 is a slow-growing tumor that usually does not spread to the lymph nodes or to other parts of the body. Most children are diagnosed with thymoma at an early stage.

Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymoma.

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

People who develop thymoma often have one of the following immune system diseases or hormone disorders:

Thymoma 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 thymoma may cause these same signs and symptoms.

Tests to diagnose and stage thymoma 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. Childhood thymoma is usually diagnosed before the tumor has spread.

Treatment

Treatment for thymoma in children may include the following:

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

Thymic Carcinoma

Thymic carcinoma is a rare cancer of the cells that cover the outside surface of the thymus. 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. Thymic carcinoma usually forms in the front part of the chest and is often found during a chest x-ray that is done for another reason.

Anatomy of the thymus gland; drawing shows the thymus gland in the upper chest under the breastbone. Also shown are the ribs, lungs, and heart.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.

Thymic carcinoma is more likely than thymoma to spread to the lymph nodes or to other parts of the body by the time it is diagnosed.

Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymic carcinoma or thymoma. (See the Thymoma section above for more information).

Treatment

Thymic carcinoma can rarely be completely removed by surgery and is likely to recur (come back) after treatment.

Treatment for 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:

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:

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:

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:

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.

Unusual Cancers of the Abdomen

Adrenocortical Carcinoma

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. Adrenocortical carcinoma is also called cancer of the adrenal cortex.

Childhood adrenocortical carcinoma 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:

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

The risk of adrenocortical carcinoma 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 breast tissue in male children. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the signs and symptoms of adrenocortical carcinoma.)

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 adrenocortical carcinoma include the following:

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:

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

Treatment

Treatment for adrenocortical carcinoma 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.

Gastrointestinal (digestive) system anatomy; drawing shows the esophagus, liver, stomach, small intestine, and 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:

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; drawing shows the pancreas, stomach, spleen, liver, gallbladder, bile ducts, colon, and small intestine. An inset shows the head, body, and tail of the pancreas. The bile duct and pancreatic duct are also shown.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:

There are four types of pancreatic cancer in children:

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:

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:

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:

Treatment

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

Treatment of pancreatoblastoma in children may include the following:

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

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).

Gastrointestinal (digestive) system anatomy; shows esophagus, liver, stomach, colon, small intestine, rectum, and anus.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 may be part of an inherited syndrome. Some colorectal cancers in young people are linked to a gene mutation 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:

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:

Prognosis

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

Treatment

Treatment for colorectal cancer in children may include the following:

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

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:

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:

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. Larger tumors are more likely to recur (come back).

Treatment

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

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:

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:

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; drawing shows a front view of the right and left kidneys, the ureters,  urethra, and bladder filled with urine. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. The spine, adrenal glands, and uterus are also shown.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:

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; drawing shows front and side views of ureters, lymph nodes, rectum, bladder, prostate gland, vas deferens,  penis, testicles, urethra, seminal vesicle, and ejaculatory duct.Anatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs.

There are two types of testicular tumors:

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:

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; drawing shows the uterus, myometrium (muscular outer layer of the uterus), endometrium (inner lining of the uterus), ovaries, fallopian tubes, cervix, and vagina.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 ovarian tumors 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:

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; drawing shows the uterus, myometrium (muscular outer layer of the uterus), endometrium (inner lining of the uterus), ovaries, fallopian tubes, cervix, and vagina.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.

Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes

Multiple endocrine neoplasia (MEN) syndromes are inherited disorders 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:

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:

Treatment

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

Pheochromocytoma and Paraganglioma

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

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 inherited syndromes 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:

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:

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:

Anatomy of the skin with melanocytes; drawing shows normal skin anatomy, including the epidermis, dermis, hair follicles, sweat glands, hair shafts, veins, arteries, fatty tissue, nerves, lymph vessels, oil glands, and subcutaneous tissue. The pullout shows a close-up of the squamous cell and basal cell layers of the epidermis above the dermis with blood vessels. Melanin is shown in the cells. A melanocyte is shown in the layer of basal cells at the deepest part of the epidermis. 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:

Signs and symptoms of melanoma include the following:

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:

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-dose interferon 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

Nonmelanoma skin cancers (squamous cell and basal cell cancers) are very rare in children and adolescents. The risk of squamous cell or basal cell cancer is increased by the following:

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

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

Treatment of Squamous Cell and Basal Cell Skin Cancer

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

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

Intraocular (Uveal) Melanoma

Intraocular melanoma begins in the middle of three layers of the wall of the eye. The outer layer includes the white sclera (the "white of the eye") and the clear cornea at the front of the eye. The inner layer has a lining of nerve tissue, called the retina, which senses light and sends images along the optic nerve to the brain. The middle layer, where intraocular melanoma forms, is called the uvea or uveal tract, and has three main parts: the iris, the ciliary body, and the choroid.

Eye anatomy; two-panel drawing shows the outside and inside of the eye. The top panel shows outside of the eye including the eyelid, pupil, sclera, and iris; the bottom panel shows inside of the eye including the cornea, lens, ciliary body, retina, choroid, optic nerve, and vitreous humor.Anatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a liquid that fills the center of the eye.

Risk Factors

The risk of intraocular melanoma is increased by any of the following:

Treatment

Treatment for intraocular melanoma in children is similar to treatment for adults and may include surgery, radiation therapy, and laser surgery.

See the PDQ summary on adult Intraocular (Uveal) Melanoma 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 condition tuberous sclerosis, a genetic disorder 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:

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.

Carcinoma of unknown primary; drawing shows a primary tumor that has spread from an unknown site to other parts of the body (the lung and the brain). An inset shows cancer cells spreading from the primary cancer, through the blood and lymph systems, to another part of the body where a metastatic tumor has formed.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.

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:

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of unusual cancers of childhood. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

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PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ Pediatric Treatment Editorial Board. PDQ Unusual Cancers of Childhood Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/childhood-cancers/patient/unusual-cancers-childhood-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389276]

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Physicians version: CDR0000062872
Date last modified: 2017-06-23