Unusual Cancers of Childhood (PDQ�)

General Information About Unusual Cancers of Childhood

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. The number of deaths from childhood cancer is less than half what it was in 1975.

Unusual cancers are so rare that most children's hospitals see fewer than two cases in a year. 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.

• 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).

• 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.

• 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.

• 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.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.

• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.

• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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 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:

• Pediatric surgeon.

• Pediatric hematologist.

• Neurosurgeon.

• Neurologist.

• Neuropathologist.

• Neuroradiologist.

• Radiation oncologist.

• Pediatric nurse specialist.

• Rehabilitation specialist.

• Endocrinologist.

• Social worker.

• Psychologist.

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.

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 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 nasalcavity (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 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 doctor if any of the following problems occur:

• Painless lumps in the neck.

• Snoring.

• Nosebleeds.

• Hearing loss.

• Problems moving the jaw.

• 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 that examine the nasal cavity and throat are used to diagnose and stage nasopharyngeal cancer. They may include:

• Physical exam and history.

• MRI of the head and neck.

• CT scan of the chest and abdomen.

• Bone scan.

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) test: A blood test to check for antibodies to the Epstein-Barr virus. Antibodies to EBV 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:

• Radiation therapy.

• Chemotherapy given before and at the same time as radiation therapy.

• A clinical trial of radiation therapy and a drug to prevent radiation therapy side effects. These may be used with or without chemotherapy given before and at the same time as the radiation therapy.

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

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

Esthesioneuroblastoma

Esthesioneuroblastoma (olfactoryneuroblastoma) is a very rare 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.

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

Esthesioneuroblastoma may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Blocked nose.

• Loss of the sense of smell.

• Frequent sinusinfections.

• Nosebleeds.

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

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:

• Surgery and radiation therapy. Newer treatments include sinus surgery done through an endoscope, radiosurgery, or proton beam radiation therapy.

• Chemotherapy and/or radiation therapy followed by surgery.

• Surgery followed by chemotherapy.

Thyroid Tumors

Thyroidtumors 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). 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.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of thyroid cancer is increased by being exposed to radiation and by certain geneticsyndromes, such as multiple endocrine neoplasia (MEN) type 2A syndrome or multiple endocrine neoplasia (MEN) type 2B syndrome.

Thyroid tumors may cause any of the following symptoms. Check with your doctor if any of the following problems occur:

• A lump in the neck.

• Trouble breathing.

• Trouble swallowing.

• Hoarseness or a change in the voice.

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

Tests that examine the thyroid are used to diagnose and stage thyroid tumors. They may include:

• Physical exam and history.

• Fine-needle aspiration (FNA) biopsy.

• Open biopsy or surgery to remove all or part of the thyroid.

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:

• Where cancer is found in the body when it is diagnosed.

• The size of the tumor.

Treatment

Treatment of thyroid tumors in children may include the following:

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

• Surgery to remove the lobe in which thyroid cancer is found, followed by HRT to make up for the lost thyroid hormone.

• Radioactive iodine (RAI) for cancer that has recurred (come back).

• Targeted therapy with tyrosine kinase inhibitors (TKIs) or vascular endothelial growth factor inhibitors (VEGFs) for cancer that has spread to other parts of the body or that has recurred.

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 Cancer

Oral cancer is a disease in which malignant (cancer) cells form in the tissues of the oral cavity. Most tumors in the oral cavity are benign (not cancer). The most common type of oral cancer in adults, squamous cell carcinoma (cancer of the thin, flat cells lining the mouth), is very rare in children. However, the number of new cases of oral cancer in teenage girls and young women has increased since the mid-1990s. Malignant tumors in children include lymphomas and sarcomas.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of oral cancer is increased by the following:

• Tobacco use: Using any tobacco product increases the risk of oral cancer. Use of smokeless tobacco may cause mouth cancer. Changes in the texture, color, and shape of tissue inside the mouth have been seen in more than half of all teenagers who use smokeless tobacco.

• Previous radiation therapy: Oral cancer is more likely in people who have had other childhood tumors and were treated with radiation therapy to the oral cavity.

• Having certain diseases or conditions, such as:

  • Fanconi anemia.

  • Dyskeratosis congenita (a rare bone marrowdisorder that affects red blood cells, white blood cells, and platelets).

  • A mutation in connexin genes (changes the way proteins that connect cells are made).

  • Chronicgraft-versus-host disease (GVHD).

  • Epidermolysis bullosa (an illness that causes the skin to be easily injured and causes painful blisters).

  • Xeroderma pigmentosum.

  • Human papillomavirus (HPV) infection.

Oral cancer may cause any of the following signs and symptoms. Check with you doctor if any of the following problems occur:

• A sore in the mouth that does not heal.

• A lump or thickening in the oral cavity.

• A white or red patch on the gums, tongue, tonsils, or lining of the mouth.

• Bleeding, pain, or numbness in the mouth.

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

Tests that examine the mouth are used to diagnose and stage oral cancer. They may include:

• Physical exam and history.

• X-ray.

• MRI of the head and neck.

• CT scan.

• PET scan.

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

Treatment

Treatment of oral cancer in children may include the following:

• Surgery for most benign tumors.

• Surgery, chemotherapy, and radiation therapy for malignant tumors.

See the following PDQ summaries for more information:

• Oropharyngeal Cancer Treatment

• Lip and Oral Cavity Cancer Treatment

• Langerhans Cell Histiocytosis Treatment

Salivary Gland Tumors

Salivary glandtumors 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). Malignant (cancer) salivary gland tumors are rare. Malignant tumors sometimes form after treatment with radiation therapy for leukemia or solid tumors.

Symptoms and Diagnostic and Staging Tests

Salivary gland tumors may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• A lump (usually painless) near the ear, cheek, jaw, or lip, or inside the mouth.

• Fluid draining from the ear.

• Trouble swallowing or opening the mouth widely.

• Numbness or weakness in the face.

• Pain in the face that does not go away.

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

Tests that examine the mouth are used to diagnose and stagesalivary gland cancer. They may include:

• Physical exam and history.

• MRI of the head and neck.

• CT scan.

• PET scan.

• Ultrasound.

• Endoscopy.

• Fine-needle aspiration (FNA) biopsy.

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 in children is usually surgery to remove the cancer, with or without radiation therapy and chemotherapy.

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 holds 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.

Symptoms and Diagnostic and Staging Tests for Laryngeal Cancer

Laryngeal cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Hoarseness or a change in the voice.

• Trouble or pain when swallowing.

• A lump in the neck or throat.

• A sore throat or cough that does not go away.

• Ear pain.

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

Tests that examine the throat and larynx are used to diagnose and stage laryngeal cancer. They may include:

• Physical exam and history.

• MRI of the head and neck.

• CT scan.

• Ultrasound.

• Endoscopy.

• Fine-needle aspiration (FNA) biopsy.

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

Other tests used to diagnose laryngeal cancer include the following:

• Laryngoscopy: A procedure in which the doctor examines the larynx (voice box) with a mirror or with a laryngoscope (a thin, lighted tube).

• Barium swallow: A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metalliccompound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.

Treatment of Laryngeal Cancer

Treatment of laryngeal cancer in children may include the following:

• Chemotherapy and radiation therapy after a biopsy, for rhabdomyosarcomas.

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

See the following PDQ summaries for more information:

• Childhood Rhabdomyosarcoma Treatment

• Laryngeal Cancer Treatment

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:

• Lasersurgery for papillomatosis and other benign tumors.

• Biologic therapy for papillomas that keep come back after being removed by surgery four times in one year.

Midline Tract Cancer with NUT Gene Changes

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

Prognosis

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

Treatment

There is no standard treatment for midline tract cancer with NUT gene changes. Taking part in a clinical trial should be considered.

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 form in both male and female children.

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, Symptoms, and Diagnostic and Staging Tests

The risk of breast cancer is increased by the following:

• Having a personal history of cancer that may spread to the breast, such as leukemia, rhabdomyosarcoma, soft tissue sarcoma, or lymphoma.

• Past treatment for another cancer, such as Hodgkin lymphoma, with radiation therapy to the breast or chest.

Breast cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• A lump or thickening in or near the breast or in the underarm area.

• A change in the size or shape of the breast.

• A dimple or puckering in the skin of the breast.

• A nipple turned inward into the breast.

• Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin that is around the nipple).

• Dimples in the breast that look like the skin of an orange, called peau d?orange.

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

Tests that examine the breast are used to diagnose and stage breast cancer. They may include:

• Physical exam and history.

• MRI.

• Ultrasound.

• PET scan.

• Blood chemistry studies.

• X-ray of the chest.

• Biopsy.

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 to check for breast cancer beginning at age 25, or 10 years after finishing radiation therapy, whichever is later.

Treatment

Treatment of breast cancer in children may include the following:

• Watchful waiting, for benign tumors.

• Surgery to remove malignant tumors, with or without radiation therapy.

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

Bronchial Tumors

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

Symptoms and Diagnostic and Staging Tests

Bronchial tumors may cause any of the following signs and symptoms:

• Coughing.

• Wheezing.

• Trouble breathing.

• Spitting up blood from the airways or lung.

Other conditions that are not bronchial tumors may cause these same 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.

Tests that examine the bronchi and lungs are used to diagnose and stage bronchial tumors. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

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:

• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A contrast dye may be put through the bronchoscope to make the larynx, trachea, and airways show up clearer on x-ray film.

• Octreotide scan: A type of radionuclide scan used to find tumors. A small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.

Prognosis

Bronchial cancer in children can usually be cured, even when it has spread to nearby areas. The prognosis (chance of recovery) depends on how the cells look under a microscope and the stage of the cancer.

Treatment

Treatment of bronchial tumors in children may include the following:

• Surgery to remove the tumor. Sometimes a type of surgery called a sleeve resection is used. The lymph nodes and vessels where cancer has spread are also removed.

• Chemotherapy or radiation therapy, for cancer that has spread to other parts of the body.

Pleuropulmonary Blastoma

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

There are three stages of PPB that are described as types:

• Type I tumors are cyst-like tumors in the lung. They are most common in children aged 2 years and younger and can usually be cured.

• Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain.

• Type III tumors are solid. These tumors often spread to the brain.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of PPB is increased by the following:

• Having cysts in the lung at birth.

• Having a family history of any type of cancer in close relatives.

• Having a brother or sister with PPB.

PPB may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• A cough that doesn?t go away.

• Trouble breathing.

• Chest discomfort.

• Wheezing.

• Streaks of blood in sputum (mucus coughed up from the lungs).

• Hoarseness.

• Pain under the rib cage.

• Pain, swelling, or lumps in the abdomen.

• Loss of appetite.

• Weight loss for no known reason.

• Feeling very tired.

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

Tests that examine the lungs and the lining around the lungs are used to diagnose and stage PPB. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• PET scan.

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

Other tests used to diagnose PPB include the following:

• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

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

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

Treatment

Treatment of pleuropulmonary blastomas in children is usually surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.

Esophageal Tumors

Esophagealtumors 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.

Symptoms and Diagnostic and Staging Tests

Esophageal cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Trouble swallowing.

• Weight loss.

• Pain behind the breastbone.

• Hoarseness and cough.

• Indigestion and heartburn.

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

Tests that examine the esophagus are used to diagnose and stage esophageal cancer. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• PET scan.

• Ultrasound.

• Biopsy.

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

Other tests used to diagnose esophageal cancer include the following:

• Esophagoscopy: A procedure to look inside the esophagus to check for abnormal areas. An esophagoscope is inserted through the mouth or nose and down the throat into the esophagus. An esophagoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A biopsy is usually done during an esophagoscopy. Sometimes a biopsy shows changes in the esophagus that are not cancer but may lead to cancer.

• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

• Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. Sometimes this procedure is used to remove part of the esophagus or lung.

• Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.

Prognosis

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

Treatment

Treatment for esophageal cancer in children may include the following:

• Surgery to remove all or part of the tumor.

• Radiation therapy given through a plastic or metal tube placed through the mouth into the esophagus.

• Chemotherapy.

Thymoma and Thymic Carcinoma

Thymomas and thymic carcinomas are tumors 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. Thymomas and thymic carcinomas usually form in the front part of the chest and are often found during a chest x-ray that is done for another reason.

Thymoma and thymic carcinoma are slow-growing cancers that may spread to the lymph nodes or to other parts of the body.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

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

• Myasthenia gravis.

• Polymyositis.

• Lupus.

• Rheumatoid arthritis.

• Thyroiditis.

• Isaac syndrome.

• Pure red cell aplasia.

• Hyperthyroidism.

• Addison disease.

• Panhypopituitarism.

Thymoma and thymic carcinoma may cause any of the following symptoms Check with your doctor if any of the following problems occur:

• Coughing.

• Trouble swallowing.

• Pain or a tight feeling in the chest.

• Trouble breathing.

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

Tests that examine the cells that cover the thymus are used to diagnose and stage thymoma and thymic carcinoma. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• PET scan.

• MRI.

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.

Treatment

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

• Surgery to remove as much of the tumor as possible, followed by radiation therapy for tumors that have spread.

• Chemotherapy.

Heart Tumors

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

• Rhabdomyoma: A tumor that forms in muscle made up of long fibers.

• Fibroma: A tumor that forms in fiber-like tissue that holds bones, muscles, and other organs in place.

• Myxoma: A tumor that may be part of an inheritedsyndrome called Carney complex. (See the Multiple Endocrine Neoplasia Syndromes section for more information.)

• Histiocytoid cardiomyopathy tumor: A tumor that forms in the heart cells that control heart rhythm.

• Teratomas: A type of germ cell tumor. In the heart, these tumors form most often in the pericardium (the sac that covers the heart). Some teratomas are malignant (cancer).

• Hemangiomas: A tumor that forms in the cells that line blood vessels.

• Neurofibroma: A tumor that forms in the cells and tissues that cover nerves.

In children, the most common benign heart tumors are rhabdomyomas and fibromas. Before birth and in newborns, the most common benign heart tumors are teratomas. An inherited disorder 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 tumors in children. Some of these include:

• Malignant teratoma.

• Rhabdomyosarcoma: A cancer that forms in muscle made up of long fibers.

• Chondrosarcoma: A type of cancer that usually forms in bone cartilage but very rarely can begin in the heart.

• Infantile fibrosarcoma.

Some cancers, such as rhabdomyosarcoma, melanoma, and leukemia, spread to the heart from other parts of the body. These tumors are malignant.

Symptoms

Heart tumors may cause any of the following symptoms. Check with your doctor if any of the following problems occur:

• Change in the heart's normal rhythm.

• Trouble breathing, particularly when you are lying down.

• Pain in the middle of the chest that feels better when you are sitting up.

• Coughing.

• Fainting.

• Feeling dizzy, tired, or weak.

• Fast heart rate.

• Swelling in the legs, ankles, or abdomen.

• Feeling anxious.

Heart tumors sometimes cause sudden death without causing any symptoms.

Other conditions that are not heart tumors may cause these same symptoms. Sometimes heart tumors do not cause any symptoms at all.

Tests that examine the cells of the heart are used to diagnose and stage heart tumors. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• MRI.

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

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

• Echocardiogram: A procedure in which high-energy sound waves (ultrasound) are bounced off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.

• Electrocardiogram (EKG): A recording of the heart's electrical activity to evaluate its rate and rhythm. A number of small pads (electrodes) are placed on the patient?s chest, arms, and legs, and are connected by wires to the EKG machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.

Treatment

Treatment for heart tumors in children may include the following:

• Watchful waiting for benign tumors of heart muscle (rhabdomyomas), which usually shrink and go away on their own.

• Surgery (which may include a heart transplant) and chemotherapy for tumors that spread to the heart from other places in the body.

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.

Risk Factors, 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 doctor if any of the following problems occur:

• Trouble breathing.

• Pain under the rib cage.

• Weight loss for no known reason.

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

Tests that examine the pleura are used to diagnose and stage mesothelioma. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• PET scan.

• Fine-needle aspiration (FNA) biopsy.

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

Other tests used to diagnose mesothelioma include the following:

• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

• Thoracoscopy: An incision (cut) is made between two ribs and a thoracoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted into the chest to check for signs of disease.

• Thoracotomy: An incision (cut) is made between two ribs to check inside the chest for signs of disease.

• Cytologic exam: An exam of cells under a microscope (by a pathologist) to check for anything abnormal. For mesothelioma, fluid is taken from around the lungs or from the abdomen. A pathologist checks the cells in the fluid.

Prognosis

The prognosis (chance of recovery) is better when the tumor has not spread or come back after treatment.

Treatment

Treatment for mesothelioma in children may include one or more of the following:

• Surgery to remove the part of the chest lining with cancer and some of the healthy tissue around it.

• Chemotherapy.

• Radiation therapy, as palliative therapy, to relieve pain and improve quality of life.

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

Unusual Cancers of the Abdomen

Cancer of the Adrenal Cortex

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

The adrenal cortex makes important hormones that do the following:

• Balance the water and salt in the body.

• Help keep blood pressure normal.

• Help control the body's use of protein, fat, and carbohydrates.

• Cause the body to have male or female characteristics.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of cancer of the adrenal cortex is increased by having any of the following syndromes:

• Li-Fraumeni syndrome.

• Beckwith-Wiedemann syndrome.

• Hemihypertrophy.

• Multiple endocrine neoplasia Type I (MEN1) syndrome.

• Familial adenomatous polyposis.

• Carney complex.

A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The 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 testosterone may cause both male and female children to develop masculine traits, such as body hair or a deep voice. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the symptoms of cancer of the adrenal cortex.)

Tests that examine the adrenal gland are used to diagnose and stage cancer of the adrenal cortex. The tests and procedures used to diagnose adrenocortical carcinoma depend on the patient's symptoms. They may include:

• Physical exam and history.

• Blood chemistry studies.

• CT scan.

• MRI.

• PET scan.

• Fine-needle aspiration (FNA) biopsy.

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

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

• Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these substances in the urine may be a sign of disease in the adrenal cortex.

• Low-dosedexamethasone suppression test: A test in which one or more small doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.

• High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.

• Blood tests: Tests to measure the levels of testosterone or estrogen in the blood. A higher than normal amount of these hormones that may be a sign of adrenocortical carcinoma.

• Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.

• Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.

Prognosis

The prognosis (chance of recovery) is good for patients who have small tumors that have been completely removed by surgery. The cancer is harder to treat when the tumor is large or when the cancer has spread to other parts of the body when it was diagnosed. These tumors can spread to the kidneys, lungs, bones, and brain.

Treatment

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

• Surgery with or without chemotherapy.

• A second surgery for tumors that come back and for tumors that spread to the inferior vena cava (the large vein that empties into the heart).

• A clinical trial of surgery with or without chemotherapy.

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.

Symptoms and Diagnostic and Staging Tests

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

• Stomach pain.

• Loss of appetite.

• Weight loss for no known reason.

• Nausea.

• Vomiting.

• Constipation or diarrhea.

• Weakness.

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

Tests that examine the stomach and esophagus are used to diagnose and stage stomach cancer. They may include:

• Physical exam and history.

• X-ray of the abdomen.

• Blood chemistry studies.

• CT scan.

• Biopsy.

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

Other tests used to diagnose stomach cancer include the following:

• Upper endoscopy: A procedure to look inside the esophagus, stomach, and duodenum (first part of the small intestine) to check for abnormal areas. An endoscope is passed through the mouth and down the throat into the esophagus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease

• Barium swallow: A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metalliccompound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.

• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

  • The number of red blood cells, white blood cells, and platelets.

  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

  • The portion of the blood sample made up of red blood cells.

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:

• Surgery to remove the cancer and some healthy tissue around it.

• Surgery to remove as much of the cancer as possible, followed by radiation therapy and/or chemotherapy.

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).

The pancreas has two main jobs in the body:

• To make juices that help digest (break down) food. These juices are secreted into the small intestine.

• To make hormones that help control the sugar and salt levels in the blood. These hormones are secreted into the bloodstream.

Symptoms and Diagnostic and Staging Tests

Most pancreatic tumors do not secrete hormones. Pancreatic tumors that do secrete hormones may cause symptoms. The symptoms depend on the type of hormone being made.

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

• Weakness.

• Feeling very tired.

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

• Coma.

Other symptoms caused by tumors that make hormones include the following:

• Watery diarrhea.

• Abnormalsodium (salt) level in the blood: Having a low sodium level can cause confusion, sleepiness, muscle weakness, and seizures. Having a high sodium level may cause weakness, tiredness, confusion, paralysis, coma, and seizures.

• A lump in the abdomen.

• Weight loss for no known reason.

• Pain in the abdomen.

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

• Jaundice (yellowing of the skin and whites of the eyes).

• Blood in the stool or vomit.

Check with your doctor if any of these problems occur. Other conditions that are not pancreatic cancer may cause these same symptoms.

Tests that examine the pancreas are used to diagnose and stage pancreatic cancer. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• MRI.

• PET scan.

• Biopsy.

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

Other tests used to diagnose pancreatic cancer include the following:

• Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.

• Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube, called a stent, may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs for cancer.

• Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body. This test is done only if ERCP cannot be done.

• Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.

• Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.

Treatment

Treatment for children with pancreatic cancer may include the following:

• Surgery to remove all or part of the pancreas and part of the small intestine.

• Chemotherapy.

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

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 first 6 feet of the large intestine are called the large bowel or colon. The last 6 inches are the rectum and the anal canal. The anal canal ends at the anus (the opening of the large intestine to the outside of the body).

Risk Factors, Symptoms, and Diagnostic and Staging Tests

Childhood colon cancer is often part of an inheritedsyndrome that causes the disease. Some colorectal cancers in young people are linked to a genemutation that causes polyps (growths in the mucous membrane that lines the colon) to form that may turn into cancer later. This gene is also linked to an increased risk of brain and livertumors. Colon polyps that form in children who do not have an inherited syndrome are not linked to an increased risk of cancer.

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 doctor if any of the following problems occur:

• Tumors of the rectum or lower colon may cause constipation or diarrhea.

• Tumors in the part of the colon on the right side of the body may cause:

  • A lump in the abdomen.

  • Weight loss for no known reason.

  • Loss of appetite.

  • Blood in the stool.

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

Tests that examine the colon and rectum are used to diagnose and stage colorectal cancer. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• PET scan.

• MRI.

• Bone scan.

• Biopsy.

Other tests used to diagnose colorectal cancer include the following:

• Colonoscopy: A procedure to look inside the rectum and colon for polyps, abnormal areas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.

• Sigmoidoscopy: A procedure to look inside the rectum and sigmoid (lower) colon for polyps, abnormal areas, or cancer. A sigmoidoscope is inserted through the rectum into the sigmoid colon. A sigmoidoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.

• Fecal occult blood test: A test to check stool (solid waste) for blood that can only be seen with a microscope. Small samples of stool are placed on special cards and returned to the doctor or laboratory for testing.

• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

  • The number of red blood cells, white blood cells, and platelets.

  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

  • The portion of the blood sample made up of red blood cells.

• Kidney function test: A test in which blood or urine samples are checked for the amounts of certain substances released by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working the way they should. This is also called a renal function test.

• Liver function test: A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.

• Carcinoembryonic antigen (CEA) assay: A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of colon cancer or other conditions.

• Barium enema: A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metalliccompound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series.

Prognosis

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

• Whether the entire tumor was removed by surgery.

• Whether the cancer has spread to other parts of the body.

Treatment

Treatment for colorectal cancer in children may include the following:

• Surgery to remove the tumor when it has not spread.

• Radiation therapy and chemotherapy for tumors in the rectum or lower colon.

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

• Colon Cancer Treatment

• Rectal Cancer Treatment

Carcinoid Tumors

Carcinoid tumors usually form in the lining of the stomach or intestines, but they can form in other organs, such as the lungs or liver. These tumors are usually small, slow-growing, and benign (not cancer). Some carcinoid tumors are malignant (cancer) and spread to other places in the body. Sometimes carcinoid 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.

Symptoms and Diagnostic and Staging Tests

Some carcinoid 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 symptoms called carcinoid syndrome. Carcinoid syndrome caused by the hormone somatostatin may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Redness and a warm feeling in the face and neck.

• A fast heartbeat.

• Trouble breathing.

• Sudden drop in blood pressure.

• Diarrhea.

Other conditions that are not carcinoid tumors may cause these same symptoms.

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

• Physical exam and history.

• Blood chemistry studies.

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

Other tests used to diagnose carcinoid tumors include the following:

• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

  • The number of red blood cells, white blood cells, and platelets.

  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

  • The portion of the blood sample made up of red blood cells.

• Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The urine sample is checked to see if it contains a hormone made by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.

Treatment

Treatment for carcinoid tumors in children may include the following:

• Surgery to remove the appendix, when the tumor is small and only in the appendix.

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

• Surgery, chemotherapy, and/or radiation therapy for tumors that have spread.

For tumors that make hormones that cause symptoms, medicine can be given to help relieve the symptoms.

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

Gastrointestinal Stromal Tumors

Gastrointestinalstromal celltumors (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 children older than 10 years.

Risk Factors 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:

• Carney triad.

• Carney-Stratakis syndrome.

• Neurofibromatosis type 1 (NF1).

• Familial GIST.

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

• Feeling tired.

• Dizziness.

• A fast or irregular heartbeat.

• Shortness of breath.

• Pale skin.

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

Treatment

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

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

• Surgery to remove the tumor.

• Watchful waiting for tumors that come back in the same place or cannot be removed, but do not cause symptoms.

• A clinical trial of targeted therapy with a tyrosine kinase inhibitor (imatinib mesylate or sunitinib) for tumors that grow and spread after initial treatment or cause symptoms.

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. The bladder stores urine until it is passed out of the body. Urine is the liquid waste that is made by the kidneys when they clean the blood. The urine passes from the two kidneys into the bladder through two tubes called ureters. When the bladder is emptied during urination, the urine goes from the bladder to the outside of the body through another tube called 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, Symptoms, and Diagnostic and Staging Tests

In teenagers who were treated with certain anticancer drugs for leukemia, the risk of bladder cancer is increased.

Bladder cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following occur:

• Blood in the urine (slightly rusty to bright red in color).

• Frequent urination or feeling the need to urinate without being able to do so.

• Pain during urination.

• Lower back pain.

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

Tests that examine the bladder are used to diagnose and stage bladder cancer. They may include:

• Physical exam and history.

• CT scan.

• Ultrasound of the bladder.

• Biopsy.

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

Other tests used to diagnose bladder cancer include the following:

• Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells.

• Urine cytology: Examination of urine under a microscope to check for abnormal cells.

• Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer is in these organs. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters, and bladder, x-rays are taken to see if there are any blockages that may be caused by a tumor.

• Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

Prognosis

In children, bladder cancer is usually low grade (not likely to spread) and the prognosis is usually good following 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.

There are two types of testicular tumors:

• Germ cell tumors: Tumors that start in sperm cells in males. Testicular germ cell tumors may be benign (not cancer) or malignant (cancer). The most common testicular germ cell tumors in young boys are benign teratomas and malignant nonseminomas.

• Non-germ cell tumors: Tumors that begin in the tissues that surround and support the testicles. These tumors may be benign or malignant. The most common malignant non-germ cell tumors are seminomas. Seminomas usually occur in young men and are rare in boys.

Symptoms and Diagnostic and Staging Tests

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

Tests that examine the testicles are used to diagnose and stage non-germ cell testicular cancer. They may include:

• Physical exam and history.

• CT scan.

• Ultrasound.

• Biopsy.

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

Treatment

Treatment for non-germ cell testicular cancer in children may be surgery.

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).

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

There are several common types of ovarian tumors:

• Germ cell tumors: Tumors that start in egg cells in females. These are the most common ovarian tumors in girls. (See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on ovarian germ cell tumors.)

• Epithelial tumors: Tumors that start in the tissue covering the ovary. These are the second most common ovarian tumors in girls.

• Stromal tumors: Tumors that begin in stromal cells, which make up tissues that surround and support the ovaries.

• Other tumors, such as Burkitt lymphoma and small cell carcinoma of the ovary (a very rare tumor).

Risk Factors, Symptoms, and Diagnostic and Staging Tests

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

• Ollier disease (a disorder that causes abnormal growth of cartilage at the end of long bones).

• Maffucci syndrome (a disorder that causes abnormal growth of cartilage at the end of long bones and of blood vessels in the skin).

• Peutz-Jeghers syndrome.

Ovarian cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Painful menstrual periods.

• A lump in the abdomen.

• Pain or swelling in the abdomen.

• Having male sex traits, such as body hair or a deep voice.

• Early signs of puberty.

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

Tests that examine the ovaries are used to diagnose and stage ovarian cancer. They may include:

• Physical exam and history.

• CT scan.

• Ultrasound.

• Biopsy.

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

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 may include the following:

• Surgery.

• Radiation therapy.

• Combination chemotherapy.

Treatment of ovarian stromal tumors may include the following:

• Surgery to remove one ovary and one fallopian tube, for early cancer.

• Surgery followed by chemotherapy for cancer that is advanced.

• Chemotherapy for cancer that has recurred (come back).

See the following PDQ summaries for more information:

• Childhood Extracranial Germ Cell Tumors Treatment

• Ovarian Epithelial Cancer Treatment

• Ovarian Germ Cell Tumors Treatment

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).

The most common symptom of cervical and vaginal cancer is bleeding from the vagina. Other conditions may also cause vaginal bleeding.

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 if this is an effective treatment.

Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes and Carney Complex

Multiple endocrine neoplasia (MEN) syndromes

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

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

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

• MEN1 syndrome is also called Werner syndrome. This syndrome can cause tumors in the parathyroid, pancreas, and pituitary glands. A diagnosis of MEN1 syndrome is usually made when tumors are found in two or three of these glands. MEN1 syndrome may also cause tumors in the adrenal glands, gastrointestinal tract, fibroustissue, and fat cells. The prognosis (chance of recovery) is usually good.

  The most common sign of MEN1 syndrome is hypercalcemia. Hypercalcemia may cause weakness, feeling very tired, nausea and vomiting, loss of appetite, being very thirsty and urinating more than usual, and constipation.

• MEN2 syndrome includes three subgroups:

  • MEN2A syndrome

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

    • Medullary thyroid cancer.

    • Pheochromocytoma (a tumor of the adrenal gland).

    • Parathyroid gland cancer.

    Symptoms of medullary thyroid cancer may include the following:

    • A lump in the neck.

    • Trouble breathing.

    • Trouble swallowing.

    • Hoarseness.

    Symptoms of pheochromocytoma may include:

    • Pain in the abdomen or chest.

    • Fast or irregular heart beat.

    • Being irritable or nervous.

    • Headache.

    Symptoms of parathyroid gland cancer may include:

    • Hypercalcemia.

    • Pain in the abdomen, side, or back that doesn't go away.

    • Pain in the bones.

    • A broken bone.

    • A lump in the neck.

    • Change in voice, such as hoarseness.

    • Trouble swallowing.

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

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

  • MEN2B syndrome

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

    • Medullary thyroid cancer.

    • Parathyroid hyperplasia.

    • Adenomas.

    • Pheochromocytoma.

    • Nerve cell tumors in the mucous membranes or other places.

  • Familial medullary carcinoma of the thyroid (FMTC)

    This type of MEN2 syndrome causes medullary thyroid cancer. A diagnosis of FMTC may be made when 2 or more family members have medullary thyroid cancer and no family members have parathyroid or adrenal gland problems.

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

• Physical exam and history.

• Blood chemistry studies.

• Ultrasound.

• MRI.

• CT scan.

• PET scan.

• Fine-needle aspiration (FNA) or surgical biopsy.

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

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

• Genetic testing: A test to analyze DNA and check for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder.

• Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may be checked for abnormal levels of thyroid-stimulating hormone (TSH). TSH is made by the pituitary gland in the brain. It stimulates the release of thyroid hormone and controls how fast follicular thyroid cells grow. The blood may also be checked for high levels of the hormone calcitonin or parathyroid hormone (PTH).

• Radioactive iodinescan (RAI scan): A procedure to find areas in the body where thyroid cancer cells may be dividing quickly. Radioactive iodine (RAI) is used because only thyroid cells take up iodine. A very small amount of RAI is swallowed, travels through the blood, and collects in thyroid tissue and thyroid cancer cells anywhere in the body. Abnormal thyroid cells take up less iodine than normal thyroid cells do. Areas that do not take up the iodine normally are called cold spots. Cold spots show up lighter in the picture made by the scan. They can be either benign (not cancer) or malignant, so a biopsy is done to find out if they are cancer.

• Sestamibi scan: A type of radionuclide scan used to find an overactive parathyroid gland. A small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity.

• Angiogram: A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into a blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.

• Venous sampling for an overactive parathyroid gland: A procedure in which a sample of blood is taken from veins near the parathyroid glands. The sample is checked to measure the amount of parathyroid hormone released into the blood by each gland. Venous sampling may be done if blood tests show there is an overactive parathyroid gland but imaging tests don?t show which one it is.

• Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

• MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma. A very small amount of radioactive material called MIBG is injected into a vein and travels through the bloodstream. Pheochromocytoma cells take up the radioactive material and are detected by a device that measures radiation.

• Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma.

• Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma.

• Pentagastrin stimulation test: A test in which blood samples are checked to measure the amount of calcitonin in the blood. Calcium gluconate and pentagastrin are injected into the blood and then several blood samples are taken over the next 5 minutes. If the level of calcitonin in the blood increases, it may be a sign of medullary thyroid cancer.

Treatment

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

• Patients with the MEN1 syndrome are treated for parathyroid, pancreatic, and pituitary tumors.

• Patients with the MEN2A syndrome usually have surgery to remove the thyroid by age 5 or earlier if genetic tests show certain gene changes. The surgery is done to diagnose cancer or to prevent cancer from forming or spreading.

• Infants with the MEN2B syndrome may have the thyroid removed to prevent cancer.

• Patients with Hirschsprung disease and certain gene changes may have the thyroid removed to prevent cancer.

• A clinical trial of targeted therapy with a tyrosine kinase inhibitor for medullary thyroid cancer.

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

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:

• Squamous cells: Thin, flat cells that form the top layer of the epidermis.

• Basal cells: Round cells under the squamous cells.

• Melanocytes: Found in the lower part of the epidermis, these cells make melanin, the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment and cause the skin to darken.

There are three types of skin cancer:

• Squamous cell skin cancer.

• Basal cell skin cancer.

• Melanoma.

Squamous Cell and Basal Cell Skin Cancer

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

• Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.

• Having a fair complexion, which includes the following:

  • Fair skin that freckles and burns easily, does not tan, or tans poorly.

  • Blue or green or other light-colored eyes.

  • Red or blond hair.

• Having actinic keratosis.

• Past treatment with radiation.

• Having a weakened immune system.

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

• A sore that does not heal.

• Areas of the skin that are:

  • Small, raised, smooth, shiny, and waxy.

  • Small, raised, and red or reddish-brown.

  • Flat, rough, red or brown, and scaly.

  • Scaly, bleeding, or crusty.

  • Similar to a scar and firm.

Tests that examine the skin are used to diagnose and stage squamous cell and basal cell skin cancer include the following:

• Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.

• Biopsy: All or part of a growth that doesn't look normal is cut from the skin and viewed under a microscope by a pathologist to check for signs of cancer. There are three main types of skin biopsies:

  • Shave biopsy: A sterile razor blade is used to ?shave-off? the growth that does not look normal.

  • Punch biopsy: A special instrument called a punch or a trephine is used to remove a circle of tissue from the growth that does not look normal.

  • Excisional biopsy: A scalpel is used to remove the entire growth.

Treatment of Squamous Cell and Basal Cell Skin Cancer

Treatment for squamous cell and basal cell cancer is usually surgery to remove the tumor.

Melanoma

Melanoma is the most common skin cancer in children. It occurs more often in children aged 10 to 19 years. Melanoma rates in the United States have slowly increased since 1975.

The risk of melanoma is increased by the following:

• Giant melanocyticnevi (large black spots, which may cover the trunk and thigh).

• Xeroderma pigmentosum.

• Certain disorders of the immune system.

• Werner syndrome.

Risk factors for melanoma in all age groups include:

• Having a fair complexion, which includes the following:

  • Fair skin that freckles and burns easily, does not tan, or tans poorly.

  • Blue or green or other light-colored eyes.

  • Red or blond hair.

• Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.

• Having a history of many blistering sunburns as a child.

• Having several large or many small moles.

• Having a family history of unusual moles (atypical nevussyndrome).

• Having a family or personal history of melanoma.

Symptoms of melanoma include the following:

• A mole that:

  • changes in size, shape, or color.

  • has irregular edges or borders.

  • is more than one color.

  • is asymmetrical (if the mole is divided in half, the 2 halves are different in size or shape).

  • itches.

  • oozes, bleeds, or is ulcerated (a hole forms in the skin when the top layer of cells breaks down and the tissue below shows through).

• Change in pigmented (colored) skin.

• Satellite moles (new moles that grow near an existing mole).

Tests that examine the skin are used to diagnose and stage melanoma. They may include:

• Physical exam and history.

• X-ray of the chest.

• CT scan.

• MRI.

• PET scan.

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

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

• Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.

• Biopsy: All or part of the abnormal-looking growth is cut from the skin and viewed under a microscope by a pathologist to see if cancer cells are present. There are 4 main types of skin biopsies:

  • Shave biopsy: A sterile razor blade is used to ?shave-off? the abnormal-looking growth.

  • Punch biopsy: A special instrument called a punch or a trephine is used to remove a circle of tissue from the abnormal-looking growth.

  • Excisional biopsy: A scalpel is used to remove the entire growth.

  • Wide local excision: A scalpel is used to remove some of the normal tissue around the area where melanoma was found, to check for cancer cells. Skin grafting may be needed to cover the area where tissue was removed.

• Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes.

• Lymph node dissection: A surgical procedure in which lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called a lymphadenectomy.

• FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This test is done to tell the difference between melanoma and melanocytic tumors of unknown metastatic potential (MELTUMP).

• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

Treatment of Melanoma

Treatment for melanoma that has spread only to lymph nodes may be surgery to remove the tumor and lymph nodes with cancer, followed by biologic therapy with high-doseinterferon alpha-2b.

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

• Chemotherapy and/or biologic therapy.

• A clinical trial of high-dose biologic therapy.

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 teenagers, chordomas develop more often in the base of the skull, making them hard to remove completely with surgery.

Symptoms

Chordoma may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

• Headache.

• Neck or back pain.

• Double vision.

• Paralysis of the muscles in the face.

• Numbness, tingling, or weakness of the arms and legs.

• A change in bowel or bladder habits.

Other conditions that are not chordoma may cause these same 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 for chordoma in children is usually surgery to remove as much of the tumor as possible, followed by radiation therapy. Proton beam radiation therapy may be used.

Cancer of Unknown Primary Site

Carcinoma of unknown primary (CUP) 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 (CUP) or occult primary tumor.

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 CUP 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 under a microscope 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:

• Excisional biopsy: The removal of an entire lump of tissue.

• Incisional biopsy: The removal of part of a lump or a sample of tissue.

• Core biopsy: The removal of tissue using a wide needle.

• Fine-needle aspiration (FNA) biopsy: The removal tissue or fluid using a thin needle.

When the type of cancer cells or tissue removed is different from the type of cancer cells expected to be found, a diagnosis of CUP 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 and symptoms, and where the cancer has spread in the body. Treatment is usually chemotherapy or radiation therapy.

To Learn More About Childhood Cancer

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

• What You Need to Know About? Cancer

• Childhood Cancers

• CureSearch for Children's Cancer

• Late Effects of Treatment for Childhood Cancer

• Adolescents and Young Adults with Cancer

• Young People with Cancer: A Handbook for Parents

• Care for Children and Adolescents with Cancer

• Understanding Cancer Series: Cancer

• Cancer Staging

• Coping with Cancer: Supportive and Palliative Care

• Cancer Library

• Information for Survivors/Caregivers/Advocates

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.

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For more information from the NCI, please write to this address:

• NCI Public Inquiries Office

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Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).

Changes to This Summary (10/14/2011)

The PDQcancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Changes were made to this summary to match those made to the health professional version.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

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 are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

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." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).