General Information
Head and Neck Cancers

Cancer of the Nose and Throat Esthesioneuroblastoma Thyroid Tumors Oral (Mouth) Cancers Salivary Gland Tumors Laryngeal Cancer and Papillomatosis Respiratory Tract Cancer with Chromosome 15 Changes

Thoracic Cancers

Breast Cancer Bronchial Tumors Pleuropulmonary Blastoma Esophageal Tumors Thymomas and Thymic Carcinoma Tumors of the Heart Mesothelioma

Abdominal Cancers

Cancer of the Adrenal Cortex Cancer of the Stomach Cancer of the Pancreas Colorectal Cancer Carcinoid Tumors Gastrointestinal Stromal Cell Tumor

Genital/Urinary Tumors

Bladder Cancer Ovarian Cancer Carcinoma of the Cervix and Vagina

Other Rare Childhood Cancers

Multiple Endocrine Neoplasia Syndrome Skin Cancer (Melanoma, Basal Cell Carcinoma, Squamous Cell Carcinoma) Chordoma Cancer of Unknown Primary Site

To Learn More About Childhood Cancer
Get More Information From NCI
Changes to This Summary (09/30/2008)
About PDQ

General Information

The tumors discussed in this summary are many and different, and the discussion is arranged in descending order from tumors of the head and neck to tumors of the urinary system and skin. All of these cancers are rare enough that most pediatric hospitals might see fewer than two cases in a year. Most of these tumors are more frequent in adults with cancer; thus, much of the information about these tumors may also be sought through sources relevant to adults with these tumors.

Head and Neck Cancers

Head and neck cancers include cancers of the nose and throat, thyroid tumors, mouth cancer, salivary gland cancer, cancer of the larynx (or voice box), and respiratory tract. These cancers are discussed below.

Cancers that start in the lining of the nasal cavity and throat are called nasopharyngeal cancers. The incidence of this tumor is approximately 1 in 100,000 persons younger than 20 years in the United States.

Nasopharyngeal cancer occurs in association with Epstein-Barr virus (EBV) infection, the virus associated with infectious mononucleosis. This cancer most frequently spreads to lymph nodes in the neck, which may alert the patient, parent, or physician to the presence of this tumor. The tumor may spread to the nose, mouth, and pharynx, causing snoring, nosebleeds, obstruction of the Eustachian tubes, or hearing loss. It may invade the base of the skull, causing cranial nerve palsy or difficulty with movements of the jaw (trismus). The cancer may spread to distant sites such as the bones, lungs, and liver.

Treatment combines the use of surgery, radiation therapy, and chemotherapy. Nasopharyngeal cancer generally has spread to bones of the skull and to lymph nodes in the neck at the time of diagnosis; thus, the principal role of surgery is to obtain adequate diagnostic material from a biopsy of the involved lymph node or the primary site. Studies show that combining chemotherapy with radiation therapy is the most effective treatment for this tumor. Refer to the PDQ summary on Nasopharyngeal Cancer Treatment ¹ for more information.

Esthesioneuroblastoma (olfactory neuroblastoma) is a very rare, small tumor that begins in the olfactory bulb (the organ responsible for the sense of smell) located in the front part of the brain. Most children have a tumor in the nose or throat at the time of diagnosis. The tumor may extend into the eyes, sinuses, and the front part of the brain. Esthesioneuroblastoma occurs more often in boys and usually appears during adolescence. The disease seldom spreads to other parts of the body. Treatment for this cancer is usually surgery and radiation therapy; chemotherapy may also be used. Newer treatments may include sinus surgery done through an endoscope, radiosurgery, or proton beam radiation therapy.

Tumors of the thyroid (a gland near the windpipe that produces thyroid hormone, which helps regulate growth and metabolism) are classified as adenomas or carcinomas. Adenomas are benign (noncancerous) growths that may cause enlargement of all or part of the gland, which extends to both sides of the neck and can be quite large. Some of these tumors may secrete hormones. Transformation to a malignant carcinoma (cancer) may occur in some cells, which then may grow and spread to lymph nodes in the neck or to the lungs.

Thyroid carcinomas are rare and occur most often in girls. This cancer usually appears as a lump or mass in the thyroid with possible swelling of the lymph glands in the neck.

Surgery is the treatment required for all thyroid tumors. This is usually removal of all or nearly all of the thyroid and nearby lymph nodes in the neck. Treatment with a radioactive form of iodine is given after surgery to destroy cancer cells and thyroid tissue that remain. After surgery and treatment with radioactive iodine, hormone replacement therapy must be given to compensate for the lost thyroid hormone. Regular checkups are required to determine whether the cancer has spread to the lungs. Patients with thyroid cancer generally have an excellent survival with relatively few side effects. Thyroid tumors that recur (come back) are usually treated with radioactive iodine. Even patients with tumor that has spread to the lungs may expect no decrease in life span after appropriate treatment. (Refer to the PDQ summary on adult Thyroid Cancer Treatment ² for more information.)

Oral cancer in children or in adolescents is extremely rare. Most oral tumors are benign (not cancer). Malignant tumors include lymphomas (often Burkitt lymphoma) and sarcomas (soft tissue tumors). Oral squamous cell carcinoma (cancer of the thin, flat cells lining the mouth) is the most common type of oral cancer in adults, but is rare in children; adolescents (teens) with oral squamous cell carcinoma should be screened for a condition called Fanconi anemia. Treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy. (Refer to the PDQ summaries on adult Oropharyngeal Cancer Treatment ³ and Lip and Oral Cavity Cancer Treatment ⁴ for more information).

Salivary glands are the parts of the mouth and throat that produce saliva. Many of the tumors in these areas arise in the parotid gland. About 15% of these tumors may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw. These tumors are most frequently noncancerous but on very rare occasions may be malignant (cancerous). Sialoblastomas are a type of salivary gland tumor found in the first months of life. They are usually benign (not cancer), but may rarely be cancerous. The malignant lesions include adenocarcinoma, undifferentiated carcinoma, acinic cell carcinomas, and mucoepidermoid carcinoma. These tumors may occur after radiation therapy and chemotherapy are given for treatment of primary leukemia or solid tumors. Complete surgical removal is the treatment of choice whenever possible, with additional use of radiation therapy and chemotherapy. Prognosis (outcome) for patients with these tumors is generally good. (Refer to the PDQ summary on adult Salivary Gland Cancer Treatment ⁵ for more information.)

Benign and especially malignant (cancerous) tumors of the larynx (voice box) are rare. Malignant tumors may be associated with benign tumors such as polyps and papillomas. These tumors may cause hoarseness, difficulty swallowing, and enlargement of the lymph nodes of the neck. Rhabdomyosarcoma (a malignant tumor of muscle tissue) is the most common malignant tumor of the larynx in the pediatric age group. Squamous cell carcinoma of the larynx should be managed with surgery and radiation. Laser surgery may be the first type of treatment used for these cancers.

Papillomatosis of the larynx is a benign overgrowth of tissues lining the larynx. It is associated with the human papillomavirus (HPV). This condition is not cancerous, but may recur after treatment. These tumors can cause hoarseness because of their association with wart -like nodules on the vocal cords; they may extend into the lung and develop into cancer in the larynx. Treatment includes laser surgery. (Refer to the PDQ summary on adult Laryngeal Cancer Treatment ⁶ for more information.)

The respiratory tract includes the nose, throat, larynx, trachea, and lungs. Respiratory tract cancer may be caused by a specific chromosome change. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Cancer may develop when part of the DNA from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. This type of cancer may appear in parts of the airway or in other places along the midline of the body, including the thymus, the area between the lungs, and the bladder. It usually cannot be cured.

Thoracic Cancers

Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumors, pleuropulmonary blastoma, esophageal tumors, thymomas and thymic carcinomas, tumors of the heart, and mesothelioma. These thoracic cancers are discussed below.

Most tumors that involve the breast during childhood are benign (noncancerous) fibroadenomas that can be watched for change without the need for biopsy. Rarely, such breast tumors will show malignant change with sudden, rapid growth. These are called phyllodes tumors, and require biopsy or surgical removal without mastectomy. Other types of breast cancer have been reported in both males and females younger than 21 years. There is an increased lifetime risk of breast cancer in patients who were treated for any cancer with radiation therapy to the chest area, including female survivors of Hodgkin lymphoma. Mammograms should start at age 25 or 10 years after radiation therapy to the chest, whichever came last. Treatment options include radiation, chemotherapy, and surgery for children and adolescents with breast cancer. Breast tumors may also occur as cancers that have spread from other types of cancer such as leukemia, rhabdomyosarcoma, other sarcomas, or lymphoma, particularly in patients infected with the human immunodeficiency virus (HIV).(Refer to the PDQ summary on adult Breast Cancer Treatment ⁷ for more information.)

Bronchial tumors (which are slow-growing cancers in the trachea or large bronchi, the large airways of the lung), are most often carcinoid tumors in children. Symptoms include cough and spitting up blood, and can sometimes be confused with asthma symptoms like wheezing, resulting in delayed diagnosis. Primary treatment is surgery to remove the tumor and lymph nodes and vessels where cancer may spread. The prognosis for most bronchial tumors in children is excellent, even when the cancer has spread to nearby areas. Rarely, carcinoid tumors develop as aggressive (fast-growing) cancers that are more likely to spread to other areas of the body by the time they are diagnosed. For bronchial carcinoid tumors, neither chemotherapy nor radiation therapy is indicated, unless evidence of metastasis (spread of cancer to other areas of the body) is documented. Cancers that begin in the epithelial cells that line the surface of the lung are rare, and tend to be advanced when they are diagnosed. The outcome for epithelial cancers of the lung depends on how the cells look under a microscope and the stage of the cancer.

Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs. Early-stage pleuropulmonary blastoma develops as a cyst -like tumor in the lung. It usually appears in the first 2 years of life and has a good prognosis. Late-stage tumors are solid and can spread to the brain. When the tumor can be completely removed by surgery at any time during treatment, the chance of recovery is improved. The tumor may recur or spread, in spite of surgical removal. Responses to chemotherapy have been reported and adjuvant chemotherapy may help prevent the tumor from coming back. Radiation may be used when the tumor cannot be surgically removed. A family history of cancer in close relatives has been noted for many young patients affected by this tumor.

Cancer of the esophagus (the muscular tube through which food passes from the throat to the stomach) is rare in the pediatric age group. Symptoms are related to difficulty in swallowing, and associated weight loss. Treatment options for esophageal cancer include either radiation therapy or chemotherapy. Prognosis generally is poor for this cancer, which rarely can be completely removed by surgery. (Refer to the PDQ summary on adult Esophageal Cancer Treatment ⁸ for more information.)

A cancer of the thymus (an organ in the chest, behind the breastbone) is not considered a thymoma (cancer) or a thymic carcinoma unless there are cancerous changes of the epithelial cells that cover the organ. The term thymoma usually describes cancers that do not have obvious changes in the epithelial cells. Thymic carcinoma has clear-cut changes of the epithelial cells. Other tumors that involve the thymus gland include lymphoma (cancer that arises in cells of the lymphatic system) and germ cell tumors (tumors that begin in cells that give rise to sperm or eggs); these tumors are not true thymomas or thymic carcinomas.

Thymomas and thymic carcinomas are rare in adults as well as children. Various diseases and syndromes are associated with thymomas, including myasthenia gravis, polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis, Isaacs syndrome, and pure red cell aplasia. Endocrine (hormonal) disorders such as hyperthyroidism, Addison’s disease, and panhypopituitarism can also be associated with a diagnosis of thymoma or thymic carcinoma.

Cancer of the thymus may be caused by a specific chromosome change. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Cancer may develop when part of the DNA from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. This type of cancer may appear in the thymus or in other places along the midline of the body, including parts of the airway, the area between the lungs, and the bladder. It usually cannot be cured.

Thymomas and thymic carcinomas are usually located in the front part of the chest and are usually discovered during a routine chest x-ray. Symptoms can include cough, difficulty with swallowing, tightness of the chest, chest pain, and shortness of breath, although nonspecific symptoms may occur. These tumors generally are slow growing but are potentially invasive, with cancer spreading to distant organs or lymph nodes. Surgery is performed with the goal of a complete removal. Radiation therapy is necessary for patients with invasive thymoma or thymic carcinoma, whether or not there has been surgery. Chemotherapy is usually reserved for patients with advanced- stage disease who have not responded to radiation therapy or steroids. The prognosis for patients with invasive thymoma or thymic carcinoma usually is poor, although significantly higher survival rates have been reported for patients with tumors that have not spread to the surrounding areas. (Refer to the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment ⁹ for more information.)

Primary tumors of the heart may include benign (noncancerous) and malignant (cancerous) teratoma (a tumor made up of a mixture of tissues), rhabdomyosarcoma (a tumor of muscle tissue), hemangioma (a usually benign tumor made up of blood vessels), and chondrosarcoma (a type of cancer that forms in cartilage). Symptoms include abnormalities of heart rhythm, enlargement of the heart, fluid in the pericardial sac, and congestive heart failure. Successful treatment requires surgery (which may include transplantation) and chemotherapy appropriate for the type of cancer that is present. Benign tumors of heart muscle (rhabdomyomas) usually shrink and go away on their own.

Mesothelioma, an extremely rare cancer in children, can involve the tissue coverings of the lung, the heart, or the abdominal organs. These tumors can spread over the surface of organs, without invading far into the underlying tissue, and may spread to nearby or distant lymph nodes. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation. In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation. The amount of exposure required to develop cancer is unknown, and there is no information about the risk of children exposed to asbestos. (Refer to the PDQ summary on adult Malignant Mesothelioma Treatment ¹⁰ for more information.)

Abdominal Cancers

Abdominal cancers include cancer of the adrenal cortex, stomach cancer, cancer of the pancreas, colorectal cancer, carcinoid tumors of the lung or intestine, and gastrointestinal stromal cell tumors. These abdominal cancers are discussed below. (Refer to the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors ¹¹ for information about childhood renal cell carcinoma.)

The adrenal cortex is the outside layer of the adrenal glands. The adrenal glands are a pair of organs near the front side edge of the kidney; their function is to produce hormones such as glucocorticoid and epinephrine. Cancers in this area are classified as carcinomas and adenomas. Adenomas are generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, regardless of the patient’s gender. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, an inherited condition that predisposes family members to multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma (cancer of the bone).

These tumors can involve the kidneys, lungs, bones and brain. Surgical removal should be attempted but may not always be possible if the tumor has spread widely. Additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumor. The prognosis is generally excellent for patients who have small tumors that have been completely removed by surgery, but prognosis can be poor for patients who have large primary tumors or metastatic disease (disease that has spread to other parts of the body) at diagnosis. Tumor stage is an important factor affecting the chance of recovery for children with adrenocortical tumors. When possible, repeat surgery should be done for tumors that come back and for tumors that spread to the inferior vena cava (a large vein that empties into the heart). (Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment ¹² for more information.)

The frequency of, and death rate from, stomach cancer has declined worldwide over the past 50 years with the introduction of food preservation practices such as refrigeration. Symptoms of stomach cancer include vague upper abdominal pain, which can be associated with poor appetite, and weight loss. Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite and weakness, and Helicobacter pylori infection.

Treatment should include surgery. For individuals who cannot have a complete surgical removal of tissue, radiation therapy may be used along with chemotherapy. Prognosis depends on the extent of the disease at the time of diagnosis and the success of treatment that is appropriate for the clinical situation. Because of the rarity of stomach cancer in the pediatric age group, little information exists regarding treatment outcomes of children. (Refer to the PDQ summary on adult Gastric Cancer Treatment ¹³ for more information.)

Tumors of the pancreas (a gland in the abdomen that makes pancreatic juices and produces hormones) are rare in children and adolescents. Tumors included within the general category can arise at any site in the pancreas. Most pancreatic tumors do not secrete hormones, although some tumors secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma. If a tumor interferes with the normal function of the islet cells (cells in the pancreas that produce hormones), patients may have watery diarrhea or abnormalities of salt balance. At times, there is obstruction of the head of the pancreas, which is associated with jaundice and gastrointestinal bleeding.

Treatment includes various surgical procedures to remove the pancreas and duodenum or part of the pancreas. For pediatric patients, the effectiveness of radiation therapy is not known. Chemotherapy may be useful for treatment of localized or metastatic pancreatic carcinoma, although few cases have been successfully treated. Pancreatoblastoma may be treated with combination chemotherapy given before or after surgery. Response rates and survival rates generally are not good. (Refer to the PDQ summary on adult Pancreatic Cancer Treatment ¹⁴ for more information.)

Cancer of the large bowel is rare in the pediatric age group: one person per one million younger than 20 years in the United States annually. In children, more than half of colon tumors begin on the right side, compared with adults, who have more colon tumors on the left side. Colon cancer in children is often linked to a family colorectal cancer syndrome, or inherited pattern. There is an increasing risk of colorectal cancer in members of families with a family history of intestinal polyps, which can lead to the development of multiple adenomatous polyps (benign tumors). Juvenile polyps are not associated with an increased incidence or risk of cancer.

Colorectal cancer usually presents with symptoms related to the site of the tumor. Changes in bowel habits are associated with tumors of the rectum or lower colon. Tumors of the right colon may cause more subtle symptoms but are often associated with an abdominal mass, weight loss, decreased appetite, and blood in the stool. Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.

Colorectal carcinoma is rarely diagnosed in a pediatric patient; however, vague gastrointestinal symptoms should alert the physician to investigate this possibility. Most patients present with evidence of metastatic disease (cancer that has spread to other body parts), either as gross tumor or as microscopic deposits in lymph nodes, on the surface of the bowel, or other organs within the abdomen. Complete surgical removal should be the primary aim of the surgeon, but in most instances, this is impossible; removal of large portions of tumor provides little benefit for the individuals with extensive metastatic disease. Most patients with microscopic metastatic disease generally develop gross metastatic disease, and few individuals with metastatic disease at diagnosis become long-term survivors.

Current therapy includes the use of radiation therapy for rectal and lower colon tumors, in conjunction with chemotherapy. (Refer to the PDQ summaries on adult Colon ¹⁵ and Rectal Cancer Treatment ¹⁶ for more information.)

Carcinoid tumors can involve the lining of the lung or the large or small bowel and may not be cancer. Most lung lesions are not cancerous. Treatment of metastatic carcinoid tumors of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal cancer. (Refer to the PDQ summary on Gastrointestinal Carcinoid Tumors Treatment ¹⁷ for more information.)

Gastrointestinal stromal cell tumor (GIST) usually begins in cells in the wall of the gastrointestinal tract. It may or may not be cancerous. This tumor is usually found in adults older than 40 years and is rare in children. GIST in children younger than 10 years is more common in girls. Symptoms of GIST include anemia caused by gastrointestinal bleeding. Most tumors in children are found in the stomach. A small number of children with GIST are found to have Carney complex, a rare, inherited disorder.Treatment of GIST is different for children than for adults, and includes surgery to remove the tumor. In children, unlike in adults, GIST is not caused by changes in DNA. When these changes are not found, treatment with imatinib mesylate (Gleevec), a new kind of cancer drug that blocks these changes, is not recommended as adjuvant treatment. Some adolescents and young adults with GIST have tumors that are caused by DNA changes and are treatable with imatinib mesylate. All patients with GIST should have their tumors examined for DNA changes.

Genital/Urinary Tumors

Genital/urinary tumors include bladder cancer, ovarian cancer, cervical cancer, and vaginal cancer. These cancers are discussed below.

Bladder cancer is extremely rare in children. The most common carcinoma to involve the bladder is transitional cell carcinoma, which generally presents with blood in the urine. The diagnosis and treatment of bladder cancer are the same for children, adolescents, and adults. Adolescents who develop this tumor are often prone to the development of other cancers. Bladder cancer in adolescents may develop as a late effect of certain chemotherapy drugs given for other childhood tumors or leukemia. (Refer to the PDQ summary on adult Bladder Cancer Treatment ¹⁸ for more information.)

Most ovarian tumors in children are benign (noncancerous). The most common cancers that affect the ovaries are of germ cell origin (beginning in cells that give rise to sperm or eggs); these are more common in children than in adults. Common symptoms of ovarian cancer include painful periods and pain in the abdomen. Treatment is stage related and may include surgery, radiation therapy, and chemotherapy. (Refer to the PDQ summaries on Childhood Extracranial Germ Cell Tumors Treatment, ¹⁹ Ovarian Epithelial Cancer Treatment ²⁰, Ovarian Germ Cell Tumors Treatment ²¹, or Ovarian Low Malignant Potential Tumors Treatment ²² for more information.)

Cancer of the cervix and vagina is very rare in children and adolescents. Most young patients diagnosed with cancer of the cervix or vagina were exposed to a drug called diethylstilbestrol (DES) before birth when it was given to their mothers. DES was given to pregnant women between 1945 and 1970 to keep them from losing their babies (miscarriage). The most common symptom of cancer of the cervix or vagina is vaginal bleeding. Treatment includes surgery followed by radiation and possibly chemotherapy.

Other Rare Childhood Cancers

Other rare childhood cancers include multiple endocrine neoplasia syndrome, skin cancer, chordoma, and cancer of unknown primary site. These other rare childhood cancers are discussed below.

Multiple endocrine neoplasia syndromes (abnormal and uncontrolled cell growth) are familial disorders that include cancerous changes in more than one endocrine organ at the same time (endocrine tissue secretes hormones). These changes may include hyperplasia (overgrowth of tissue) or benign (noncancerous) tumors.

The distinct involvement of multiple glandular structures are referred to as MEN-1 (Werner syndrome) which may involve tumors of the pituitary gland and parathyroid, adrenal, gastric, and pancreatic structures. Patients and family members at risk for MEN-1 should have both genetic screening and physical examinations.

MEN-2a (Sipple syndrome) is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma; MEN-2b is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma, mucosal neuromas, and ganglioneuromas. An additional complex is referred to as the Carney complex, which is associated with benign tumors of the heart, skin, breast, head or neck.

Patients with the MEN 2a syndrome should have surgery to remove the thyroid between the ages of 5 and 8 in order to prevent cancer. Family members of patients with the MEN 2a syndrome should be tested in early childhood, before age 5, for the abnormal gene that leads to this type of cancer. If they are found to have this genetic change, they should have surgery to remove the thyroid and replace it with a transplant of one of their own parathyroid glands by a certain age, depending on the type of genetic change found.

Patients with the MEN 2b syndrome may have a slender body build, long and thin extremities, a high arch palate, and “funnel chest” (sunken chest) or an abnormally high arch in the foot. The lips may appear thickened because of tumors in the mucous membranes. In this syndrome, medullary thyroid cancer may be particularly aggressive; therefore, the thyroid should be removed in infancy to prevent cancer in these affected children.

Hirschsprung disease (a condition present at birth that causes blockage of the large intestine due to lack of muscle contractions) is also associated with medullary thyroid carcinoma. Patients with Hirschsprung disease should be screened for genetic changes that may be linked to this cancer. If such changes are found, surgery to remove the thyroid in order to prevent cancer may be considered.

The outcome for patients with the MEN-1 syndrome is generally good, provided adequate treatment can be obtained for parathyroid, pancreatic, and pituitary tumors. The outcome for patients with the MEN-2a syndrome is also generally good, but the possibility exists for recurrence of medullary thyroid carcinoma and pheochromocytoma. Medullary thyroid cancer in children with MEN-2b may be difficult to cure. For patients with the Carney complex, prognosis depends on how often heart, skin, or other tumors recur.

Treatment of medullary thyroid carcinoma may include a clinical trial of a tyrosine kinase inhibitor, a drug that interferes with cell communication and growth and may prevent tumor growth, taken by mouth.

Melanoma is thought to be the most common skin cancer in children, followed by basal cell and squamous cell carcinomas. The incidence of melanoma in children and adolescents represents approximately 1% of the new cases of melanoma that are diagnosed annually in the United States. Melanoma is most often found in children aged 10 years or older, and is slightly more often found in girls than boys. In most instances, melanoma in the pediatric population is similar to that of adults in relation to site of presentation, symptoms, description, and spread, although thickness does not seem to predict how likely it is to be cured. Melanoma may grow faster in children than in adults. In most children and adolescents, melanoma is found as localized cancer with no sign of spread and can usually be cured. In children and adolescents with melanoma that has spread to the lymph nodes or distant parts of the body, the prognosis appears to better than the prognosis for adults with melanoma in similar stages. The reason for this difference is not known.

The most common cause of skin cancer of any type is exposure to the ultraviolet (UV) portion of sunlight. Other causes may be related to chemical carcinogenesis, radiation exposure, immunodeficiency, or immunosuppression. The person who is most likely to develop a melanoma is easily sunburned, has poor tanning ability, and generally has light hair, blue eyes, and pale skin. Worldwide, there is an increasing incidence of both melanoma and nonmelanoma skin cancers. Melanoma presents as a relatively flat, dark-colored lesion that may enlarge, penetrate the skin, or metastasize.

Melanomas may be congenital (present at birth). They are sometimes associated with large congenital black spots known as melanocytic nevi, which may cover the trunk and thigh. Children with hereditary immunodeficiencies have an increased lifetime risk of developing melanoma.

Individuals with atypical moles, which include raised lesions (that may or may not bleed) and various color hues (brown, tan, pink, black) are at an increased risk of having melanoma and having children affected by these premalignant lesions. Basal cell carcinoma generally appears as a raised lump or ulcerated lesion, usually in areas with previous sun exposure. Squamous cell carcinomas are usually reddened lesions with varying degrees of scaling or crusting; they have an appearance similar to eczema, infections, trauma, or psoriasis.

Basal and squamous cell carcinomas are generally curable with surgery alone, but the treatment of melanoma requires greater consideration because of its potential for metastasis. Surgery for melanoma depends on the size, site, level of invasion, and metastatic extent or stage of the tumor. If the melanoma has not spread beyond the lymph nodes, adjuvant biologic therapy may be given to increase the chances of a cure. If the melanoma has spread beyond the lymph nodes, treatment may include a combination of chemotherapy and biologic therapy. There are clinical trials for melanoma available to children aged 10 years or older. Information about ongoing clinical trials is available from the NCI Web site ²³.

Chordoma is a very rare type of bone tumor that may develop along the spine at any point from the base of the skull to the tailbone. Chordomas start in clusters of cells leftover from spinal column development in the embryo. These cells normally disappear, but very rarely they remain and grow into tumors. In children and adolescents, especially girls, chordomas often develop in the clivus, a bone at the base of the skull. Symptoms vary and may include pain and nerve trouble. When chordoma recurs, it usually comes back in the same area, but may appear in the lungs or other areas of bone.

Standard treatment includes surgery and radiation therapy. The best results are seen with proton beam therapy, a special kind of high-energy radiation that is different from an x-ray.

Cancer can form in any tissue of the body and can spread from the primary site (the place where the cancer first began to grow) to other parts of the body. Cancer that has spread from the place in which it started to other parts of the body is called metastatic cancer. Metastatic cancer cells usually look like cells in the type of tissue where the cancer began. For example, breast cancer cells that spread to the lung look like breast cancer cells, not lung cancer cells. Sometimes metastatic cancer cells are found in the body, but tests do not find a primary tumor. If cancer cells are found in the body but the place where the cancer started cannot be identified, the disease is called cancer of unknown primary site. Treatment is based on what the cancer cells look like under a microscope, the patient's symptoms, and the extent of the cancer 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 - An Overview ²⁴
• Childhood Cancers ²⁵
• CureSearch - National Childhood Cancer Foundation Children’s Oncology Group ²⁶
• Late Effects of Treatment for Childhood Cancer ²⁷
• Young People with Cancer: A Handbook for Parents ²⁸
• Care for Children and Adolescents with Cancer: Questions and Answers ²⁹
• Understanding Cancer Series: Cancer ³⁰
• Staging: Questions and Answers ³¹
• Coping with Cancer ³²
• Support and Resources ³³
• Cancer Library ³⁴
• Information for Survivors/Caregivers/Advocates ³⁵

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Changes to This Summary (09/30/2008)

The PDQ cancer 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 ³⁷. 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 ³⁹. 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), TTY at 1-800-332-8615.

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 ³⁷ or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

Cancer that begins in cells that line certain internal organs and that have gland-like (secretory) properties.

A noncancerous tumor that starts in gland-like cells of the epithelial tissue (thin layer of tissue that covers organs, glands, and other structures within the body).

Treatment given after the primary treatment to increase the chances of a cure. Adjuvant therapy may include chemotherapy, radiation therapy, hormone therapy, or biological therapy.

A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.

Having to do with or made by the outer layer of the adrenal gland, which produces steroid hormones. There is an adrenal gland on top of each kidney.

A quickly growing cancer.

A condition in which the number of red blood cells is below normal.

A disease that causes inflammation and pain in the joints.

A group of minerals that take the form of tiny fibers. Asbestos has been used as insulation against heat and fire in buildings. Loose asbestos fibers breathed into the lungs can cause several serious diseases, including lung cancer and malignant mesothelioma (cancer found in the lining of the lungs, chest, or abdomen). Asbestos that is swallowed may cause cancer of the gastrointestinal tract.

A chronic disease in which the bronchial airways in the lungs become narrowed and swollen, making it difficult to breathe. Symptoms include wheezing, coughing, tightness in the chest, shortness of breath, and rapid breathing. An attack may be brought on by pet hair, dust, smoke, pollen, mold, exercise, cold air, or stress.

A small, round cell found in the lower part (or base) of the epidermis, the outer layer of the skin.

A type of skin cancer that arises from the basal cells, small round cells found in the lower part (or base) of the epidermis, the outer layer of the skin.

Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body.

A noncancerous growth that does not invade nearby tissue or spread to other parts of the body.

A substance that is made from a living organism or its products and is used in the prevention, diagnosis, or treatment of cancer and other diseases. Biologic agents include antibodies, interleukins, and vaccines. Also called biological agent or biological drug.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

The organ that stores urine.

Cancer that forms in tissues of the bladder (the organ that stores urine). Most bladder cancers are transitional cell carcinomas (cancer that begins in cells that normally make up the inner lining of the bladder). Other types include squamous cell carcinoma (cancer that begins in thin, flat cells) and adenocarcinoma (cancer that begins in cells that make and release mucus and other fluids). The cells that form squamous cell carcinoma and adenocarcinoma develop in the inner lining of the bladder as a result of chronic irritation and inflammation.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The bowel has two parts, the small bowel and the large bowel. Also called the intestine.

Glandular organ located on the chest. The breast is made up of connective tissue, fat, and breast tissue that contains the glands that can make milk. Also called mammary gland.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

The large air passages that lead from the trachea (windpipe) to the lungs.

Having to do with the bronchi, which are the larger air passages of the lungs, including those that lead from the trachea (windpipe) to the lungs and those within the lungs.

Cancer that forms in tissues of the bronchi (large air passages in the lungs including those that lead to the lungs from the windpipe).

An aggressive (fast-growing) type of B-cell non-Hodgkin lymphoma that occurs most often in children and young adults. The disease may affect the jaw, central nervous system, bowel, kidneys, ovaries, or other organs. There are three main types of Burkitt lymphoma (sporadic, endemic, and immunodeficiency related). Sporadic Burkitt lymphoma occurs throughout the world, and endemic Burkitt lymphoma occurs in Africa. Immunodeficiency-related Burkitt lymphoma is most often seen in AIDS patients.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

A rare cancer that forms in the outer layer of tissue of the adrenal gland (a small organ on top of each kidney that makes steroid hormones, adrenaline, and noradrenaline to control heart rate, blood pressure, and other body functions). Also called adrenocortical cancer and adrenocortical carcinoma.

A case in which cancer cells are found in the body, but the place where the cells first started growing (the origin or primary site) cannot be determined. Also called carcinoma of unknown primary and CUP.

The process by which normal cells are transformed into cancer cells.

A slow-growing type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors may spread to the liver or other sites in the body, and they may secrete substances such as serotonin or prostaglandins, causing carcinoid syndrome.

Cancer that begins in the skin or in tissues that line or cover internal organs.

A rare, inherited disorder marked by dark spots on the skin and tumors in the heart, endocrine glands, skin, and nerves. There are two types of Carney complex, which are caused by mutations (changes) in different genes.

A tough, flexible tissue that lines joints and gives structure to the nose, ears, larynx, and other parts of the body.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Cancer that forms in tissues of the cervix (the organ connecting the uterus and vagina). It is usually a slow-growing cancer that may not have symptoms but can be found with regular Pap tests (a procedure in which cells are scraped from the cervix and looked at under a microscope).

The lower, narrow end of the uterus that forms a canal between the uterus and vagina.

Treatment with drugs that kill cancer cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

A type of cancer that forms in bone cartilage. It usually starts in the pelvis (between the hip bones), the shoulder, the ribs, or at the ends of the long bones of the arms and legs. A rare type of chondrosarcoma called extraskeletal chondrosarcoma does not form in bone cartilage. Instead, it forms in the soft tissues of the upper part of the arms and legs. Chondrosarcoma can occur at any age but is more common in people older than 40 years. It is a type of bone cancer.

A type of bone cancer that usually starts in the lower spinal cord.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

Having to do with the examination and treatment of patients.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called a clinical study.

Cancer that forms in the tissues of the colon (the longest part of the large intestine). Most colon cancers are adenocarcinomas (cancers that begin in cells that make and release mucus and other fluids).

Having to do with the colon or the rectum.

Cancer that develops in the colon (the longest part of the large intestine) and/or the rectum (the last several inches of the large intestine before the anus).

Treatment using more than one anticancer drug.

Weakness of the heart muscle that leads to a buildup of fluid in body tissues.

To heal or restore health; a treatment to restore health.

A sac or capsule in the body. It may be filled with fluid or other material.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Frequent and watery bowel movements.

DES. A synthetic form of the hormone estrogen that was prescribed to pregnant women between about 1940 and 1971 because it was thought to prevent miscarriages. DES may increase the risk of uterine, ovarian, or breast cancer in women who took it. DES also has been linked to an increased risk of clear cell carcinoma of the vagina or cervix in daughters exposed to DES before birth. Also called DES.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

Deoxyribonucleic acid. The molecules inside cells that carry genetic information and pass it from one generation to the next. Also called deoxyribonucleic acid.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

The first part of the small intestine that connects to the stomach.

A term used to describe cancer that is early in its growth, and may not have spread to other parts of the body. What is called early stage may differ between cancer types.

Early stage in the development of a plant or an animal. In vertebrate animals (have a backbone or spinal column), this stage lasts from shortly after fertilization until all major body parts appear. In particular, in humans, this stage lasts from about 2 weeks after fertilization until the end of the seventh or eighth week of pregnancy.

A thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may have a tool to remove tissue.

A hormone and neurotransmitter. Also called adrenaline.

Refers to the cells that line the internal and external surfaces of the body.

A common virus that remains dormant in most people. It causes infectious mononucleosis and has been associated with certain cancers, including Burkitt lymphoma, immunoblastic lymphoma, and nasopharyngeal carcinoma. Also called EBV.

Cancer that forms in tissues lining the esophagus (the muscular tube through which food passes from the throat to the stomach). Two types of esophageal cancer are squamous cell carcinoma (cancer that begins in flat cells lining the esophagus) and adenocarcinoma (cancer that begins in cells that make and release mucus and other fluids).

The muscular tube through which food passes from the throat to the stomach.

A record of a person's current and past illnesses, and those of his or her parents, brothers, sisters, children, and other family members. A family history shows the pattern of certain diseases in a family, and helps to determine risk factors for those and other diseases.

A rare inherited disorder in which the bone marrow does not make blood cells. It is usually diagnosed in children between 2 and 15 years old. Symptoms include frequent infections, easy bleeding, and extreme tiredness. People with Fanconi anemia may have a small skeleton and brown spots on the skin. They also have an increased risk of developing certain types of cancer.

A condition marked by extreme tiredness and inability to function due lack of energy. Fatigue may be acute or chronic.

Liquid.

Having to do with the stomach.

GI. Refers to the stomach and intestines. Also called GI.

GIST. A type of tumor that usually begins in cells in the wall of the gastrointestinal tract. It can be benign or malignant. Also called GIST.

The stomach and intestines. The gastrointestinal tract is part of the digestive system, which also includes the salivary glands, mouth, esophagus, liver, pancreas, gallbladder, and rectum.

The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

A reproductive cell of the body. Germ cells are egg cells in females and sperm cells in males.

A type of tumor that begins in the cells that give rise to sperm or eggs. Germ cell tumors can occur almost anywhere in the body and can be either benign or malignant.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

A compound that belongs to the family of compounds called corticosteroids (steroids). Glucocorticoids affect metabolism and have anti-inflammatory and immunosuppressive effects. They may be naturally produced (hormones) or synthetic (drugs).

H. pylori. A type of bacterium that causes inflammation and ulcers in the stomach or small intestine. People with H. pylori infections may be more likely to develop cancer in the stomach, including MALT (mucosa-associated lymphoid tissue) lymphoma. Also called H. pylori.

Transmitted from parent to child by information contained in the genes.

A cancer of the immune system that is marked by the presence of a type of cell called the Reed-Sternberg cell. The two major types of Hodgkin lymphoma are classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. Other symptoms include fever, weight loss, fatigue, or night sweats. Also called Hodgkin disease.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

HRT. Hormones (estrogen, progesterone, or both) given to women after menopause to replace the hormones no longer produced by the ovaries. Also called HRT and menopausal hormone therapy.

HIV. The cause of acquired immunodeficiency syndrome (AIDS). Also called HIV.

A member of a family of viruses that can cause abnormal tissue growth (for example, genital warts) and other changes to cells. Infection with certain types of human papillomavirus increases the risk of developing cervical cancer. Also called HPV.

An abnormal increase in the number of cells in an organ or tissue.

Too much thyroid hormone. Symptoms include weight loss, chest pain, cramps, diarrhea, and nervousness. Also called overactive thyroid.

Abnormally low blood sugar.

A drug used to treat different types of leukemia and other cancers of the blood, gastrointestinal stromal tumors, skin tumors called dermatofibrosarcoma protuberans, and a rare condition called systemic mastocytosis. It is also being studied in the treatment of other types of cancer. Imatinib mesylate blocks the protein made by the bcr/abl oncogene. It is a type of tyrosine kinase inhibitor. Also called Gleevec and STI571.

The decreased ability of the body to fight infections and other diseases.

Suppression of the body's immune system and its ability to fight infections and other diseases. Immunosuppression may be deliberately induced with drugs, as in preparation for bone marrow or other organ transplantation, to prevent rejection of the donor tissue. It may also result from certain diseases such as AIDS or lymphoma or from anticancer drugs.

The number of new cases of a disease diagnosed each year.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

A large vein that empties into the heart. It carries blood from the legs and feet and from organs in the abdomen and pelvis.

Transmitted through genes that have been passed from parents to their offspring (children).

A hormone made by the islet cells of the pancreas. Insulin controls the amount of sugar in the blood by moving it into the cells, where it can be used by the body for energy.

Having to do with the intestines.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The intestine has two parts, the small intestine and the large intestine. Also called the bowel.

Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.

An element that is necessary for the body to make thyroid hormone. It is found in shellfish and iodized salt.

A pancreatic cell that produces hormones (e.g., insulin and glucagon) that are secreted into the bloodstream. These hormones help control the level of glucose (sugar) in the blood. Also called endocrine pancreas cell and islet of Langerhans cell.

A condition in which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool becomes lighter than normal. Jaundice occurs when the liver is not working properly or when a bile duct is blocked.

One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.

The long, tube-like organ that is connected to the small intestine at one end and the anus at the other. The large intestine has four parts: cecum, colon, rectum, and anal canal. Partly digested food moves through the cecum into the colon, where water and some nutrients and electrolytes are removed. The remaining material, solid waste called stool, moves through the colon, is stored in the rectum, and leaves the body through the anal canal and anus.

The area of the throat containing the vocal cords and used for breathing, swallowing, and talking. Also called voice box.

A surgical procedure that uses the cutting power of a laser beam to make bloodless cuts in tissue or to remove a surface lesion such as a tumor.

Side effects of cancer treatment that appear months or years after treatment has ended. Late effects include physical and mental problems and second cancers.

A term used to describe cancer that is far along in its growth, and has spread to the lymph nodes or other places in the body.

An area of abnormal tissue. A lesion may be benign (noncancerous) or malignant (cancerous).

Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.

A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

Restricted to the site of origin, without evidence of spread.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

Cancer that forms in tissues of the lung, usually in the cells lining air passages. The two main types are small cell lung cancer and non-small cell lung cancer. These types are diagnosed based on how the cells look under a microscope.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph glands filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph node.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

An x-ray of the breast.

Surgery to remove the breast (or as much of the breast tissue as possible).

Cancer that develops in C cells of the thyroid. The C cells make a hormone (calcitonin) that helps maintain a healthy level of calcium in the blood.

A cell in the skin and eyes that produces and contains the pigment called melanin.

A form of cancer that begins in melanocytes (cells that make the pigment melanin). It may begin in a mole (skin melanoma), but can also begin in other pigmented tissues, such as in the eye or in the intestines.

A benign (noncancerous) or malignant (cancerous) tumor affecting the lining of the chest or abdomen. Exposure to asbestos particles in the air increases the risk of developing malignant mesothelioma.

The total of all chemical changes that take place in a cell or an organism. These changes make energy and the materials needed for growth, reproduction, and maintaining health. They also help get rid of toxic substances.

The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).

To spread from one part of the body to another. When cancer cells metastasize and form secondary tumors, the cells in the metastatic tumor are like those in the original (primary) tumor.

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

Cancer that has spread from the place in which it started to other parts of the body.

Too small to be seen without a microscope.

A benign (noncancerous) growth on the skin that is formed by a cluster of melanocytes (cells that make a substance called melanin, which gives color to skin and eyes). A mole is usually dark and may be raised from the skin. Also called nevus.

The moist, inner lining of some organs and body cavities (such as the nose, mouth, lungs, and stomach). Glands in the mucosa make mucus (a thick, slippery fluid). Also called mucous membrane.

The moist, inner lining of some organs and body cavities (such as the nose, mouth, lungs, and stomach). Glands in the mucous membrane make mucus (a thick, slippery fluid). Also called mucosa.

An inherited condition that may result in the development of cancers of the endocrine system. There are several types of multiple endocrine neoplasia syndrome, and patients with each type may develop different types of cancer. The altered genes that cause each type can be detected with a blood test. Also called MEN syndrome.

A rare, inherited disorder that affects the endocrine glands and can cause tumors in the parathyroid and pituitary glands and the pancreas. These tumors (usually benign) cause the glands to secrete high levels of hormones, which can lead to other medical problems, such as kidney stones, fertility problems, and severe ulcers. In some cases, tumors inside the pancreas can become cancerous. Also called MEN1 syndrome, multiple endocrine adenomatosis, and Wermer syndrome.

By or having to do with the nose.

The upper part of the throat behind the nose. An opening on each side of the nasopharynx leads into the ear.

A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

Cancer that arises in immature nerve cells and affects mostly infants and children.

A tumor that arises in nerve cells.

A benign (noncancerous) growth on the skin that is formed by a cluster of melanocytes (cells that make a substance called melanin, which gives color to skin and eyes). A nevus is usually dark and may be raised from the skin. Also called mole.

A growth or lump that may be cancerous or noncancerous.

Skin cancer that forms in basal cells or squamous cells but not in melanocytes (pigment-producing cells of the skin).

Blockage of a passageway.

Cancer that forms in tissues of the lip or mouth. This includes the front two thirds of the tongue, the upper and lower gums, the lining inside the cheeks and lips, the bottom of the mouth under the tongue, the bony top of the mouth, and the small area behind the wisdom teeth.

A part of the body that performs a specific function. For example, the heart is an organ.

A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.

Having to do with the ovaries, the female reproductive glands in which the ova (eggs) are formed. The ovaries are located in the pelvis, one on each side of the uterus.

Cancer that forms in tissues of the ovary (one of a pair of female reproductive glands in which the ova, or eggs, are formed). Most ovarian cancers are either ovarian epithelial carcinomas (cancer that begins in the cells on the surface of the ovary) or malignant germ cell tumors (cancer that begins in egg cells).

One of a pair of female reproductive glands in which the ova, or eggs, are formed. The ovaries are located in the pelvis, one on each side of the uterus.

The roof of the mouth. The front portion is bony (hard palate), and the back portion is muscular (soft palate).

A glandular organ located in the abdomen. It makes pancreatic juices, which contain enzymes that aid in digestion, and it produces several hormones, including insulin. The pancreas is surrounded by the stomach, intestines, and other organs.

Having to do with the pancreas.

A disease in which malignant (cancer) cells are found in the tissues of the pancreas. Also called exocrine cancer.

Fluid made by the pancreas. Pancreatic juices contain proteins called enzymes that aid in digestion.

One of four pea-sized glands found on the surface of the thyroid. The parathyroid hormone made by these glands increases the calcium level in the blood.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Having to do with children.

The hollow tube inside the neck that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). The pharynx is about 5 inches long, depending on body size. Also called the throat.

Tumor that forms in the center of the adrenal gland (gland located above the kidney) that causes it to make too much adrenaline. Pheochromocytomas are usually benign (noncancerous) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.

A type of tumor found in breast or prostate tissue. It is often large and bulky and grows quickly. It may be benign (not cancer) or malignant (cancer) and may spread to other parts of the body. Also called cystosarcoma phyllodes and CSP.

An exam of the body to check for general signs of disease.

Medical doctor.

The main endocrine gland. It produces hormones that control other glands and many body functions, especially growth.

A rare and very aggressive (fast-growing) cancer that forms in tissues of the lung and pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity). Pleuropulmonary blastoma is most common in children.

A growth that protrudes from a mucous membrane.

A term used to describe a condition that may (or is likely to) become cancer. Also called precancerous.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A type of high-energy, external radiation therapy that uses streams of protons (small, positively charged particles) that come from a special machine. Proton beam radiation is different from x-ray radiation.

A chronic disease of the skin marked by red patches covered with white scales.

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

Giving off radiation.

A type of external radiation therapy that uses special equipment to position the patient and precisely give a single large dose of radiation to a tumor. It is used to treat brain tumors and other brain disorders that cannot be treated by regular surgery. It is also being studied in the treatment of other types of cancer. Also called stereotaxic radiosurgery, stereotactic radiosurgery, and radiation surgery.

To become well and healthy again.

By or having to do with the rectum. The rectum is the last several inches of the large intestine closest to the anus.

To come back or to return.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrent cancer.

The organs that are involved in breathing. These include the nose, throat, larynx, trachea, bronchi, and lungs. Also called respiratory system.

In medicine, an improvement related to treatment.

The percentage of patients whose cancer shrinks or disappears after treatment.

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

The watery fluid in the mouth made by the salivary glands. Saliva moistens food to help digestion and it helps protect the mouth against infections.

A gland in the mouth that produces saliva.

A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.

Checking for disease when there are no symptoms. Since screening may find diseases at an early stage, there may be a better chance of curing the disease. Examples of cancer screening tests are the mammogram (breast), colonoscopy (colon), Pap smear (cervix), and PSA blood level and digital rectal exam (prostate). Screening can also include checking for a person’s risk of developing an inherited disease by doing a genetic test.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

Cancer that forms in tissues of the skin. There are several types of skin cancer. Skin cancer that forms in melanocytes (skin cells that make pigment) is called melanoma. Skin cancer that forms in basal cells (small, round cells in the base of the outer layer of skin) is called basal cell carcinoma. Skin cancer that forms in squamous cells (flat cells that form the surface of the skin) is called squamous cell carcinoma. Skin cancer that forms in neuroendocrine cells (cells that release hormones in response to signals from the nervous system) is called neuroendocrine carcinoma of the skin. Most skin cancers form in older people on parts of the body exposed to the sun or in people who have weakened immune systems.

The part of the digestive tract that is located between the stomach and the large intestine.

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

An abnormal mass of tissue that usually does not contain cysts or liquid areas. Solid tumors may be benign (not cancerous), or malignant (cancerous). Different types of solid tumors are named for the type of cells that form them. Examples of solid tumors are sarcomas, carcinomas, and lymphomas. Leukemias (cancers of the blood) generally do not form solid tumors.

The male reproductive cell, formed in the testicle. A sperm unites with an egg to form an embryo.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spinal column encloses the spinal cord and the fluid surrounding the spinal cord. Also called spine, backbone, and vertebral column.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column.

Cancer that begins in squamous cells, which are thin, flat cells that look like fish scales. Squamous cells are found in the tissue that forms the surface of the skin, the lining of the hollow organs of the body, and the passages of the respiratory and digestive tracts. Also called epidermoid carcinoma.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called standard of care or best practice.

A type of drug used to relieve swelling and inflammation. Some steroid drugs may also have antitumor effects.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

Cancer that forms in tissues lining the stomach. Also called gastric cancer.

The material in a bowel movement. Stool is made up of food that was not digested, bacteria, mucus, and cells from the intestines. Also called feces.

A doctor who removes or repairs a part of the body by operating on the patient.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

The percentage of people in a study or treatment group who are alive for a certain period of time after they were diagnosed with or treated for a disease, such as cancer. The survival rate is often stated as a five-year survival rate, which is the percentage of people in a study or treatment group who are alive five years after diagnosis or treatment. Also called overall survival rate.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

A chronic, inflammatory, connective tissue disease that can affect the joints and many organs, including the skin, heart, lungs, kidneys, and nervous system. It can cause many different symptoms; however, not everyone with systemic lupus erythematosus has all of the symptoms. Also called lupus and SLE.

A type of germ cell tumor that may contain several different types of tissue, such as hair, muscle, and bone. Teratomas occur most often in the ovaries in women, the testicles in men, and the tailbone in children. Not all teratomas are malignant.

Treatment.

Having to do with the chest.

The hollow tube inside the neck that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). The throat is about 5 inches long, depending on body size. Also called the pharynx.

A rare type of thymus gland cancer. It usually spreads, has a high risk of recurrence, and has a poor survival rate. Thymic carcinoma is divided into subtypes, depending on the types of cells in which the cancer began. Also called type C thymoma.

A tumor of the thymus, an organ that is part of the lymphatic system and is located in the chest, behind the breastbone.

An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.

A gland located beneath the voice box (larynx) that makes thyroid hormone and calcitonin. The thyroid helps regulate growth and metabolism.

A hormone that affects heart rate, blood pressure, body temperature, and weight. Thyroid hormone is made by the thyroid gland and can also be made in the laboratory.

A group or layer of cells that work together to perform a specific function.

The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called windpipe.

In medicine, the change that a normal cell undergoes as it becomes malignant.

Cancer that forms in transitional cells in the lining of the bladder, ureter, or renal pelvis (the part of the kidney that collects, holds, and drains urine). Transitional cells are cells that can change shape and stretch without breaking apart.

The replacement of tissue with tissue from the person’s own body or from another person.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A drug that interferes with cell communication and growth and may prevent tumor growth. Some tyrosine kinase inhibitors are used to treat cancer.

The formation of a break on the skin or on the surface of an organ. An ulcer forms when the surface cells die and are cast off. Ulcers may be associated with cancer and other diseases.

Invisible rays that are part of the energy that comes from the sun. UV radiation also comes from sun lamps and tanning beds. UV radiation can damage the skin and cause melanoma and other types of skin cancer. UV radiation that reaches the Earth's surface is made up of two types of rays, called UVA and UVB rays. UVB rays are more likely than UVA rays to cause sunburn, but UVA rays pass deeper into the skin. Scientists have long thought that UVB radiation can cause melanoma and other types of skin cancer. They now think that UVA radiation also may add to skin damage that can lead to skin cancer and cause premature aging. For this reason, skin specialists recommend that people use sunscreens that reflect, absorb, or scatter both kinds of UV radiation. Also called UV radiation.

A term used to describe cells or tissues that do not have specialized ("mature") structures or functions. Undifferentiated cancer cells often grow and spread quickly.

Having to do with urine or the organs of the body that produce and get rid of urine.

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

The muscular canal extending from the uterus to the exterior of the body. Also called birth canal.

Having to do with the vagina (the birth canal).

Cancer that forms in the tissues of the vagina (birth canal). The vagina leads from the cervix (the opening of the uterus) to the outside of the body. The most common type of vaginal cancer is squamous cell carcinoma, which starts in the thin, flat cells lining the vagina. Another type of vaginal cancer is adenocarcinoma, cancer that begins in glandular cells in the lining of the vagina.

A blood vessel that carries blood to the heart from tissues and organs in the body.

In medicine, a very simple microorganism that infects cells and may cause disease. Because viruses can multiply only inside infected cells, they are not considered to be alive.

One of two small bands of muscle within the larynx that vibrates to produce the voice.

To eject some or all of the contents of the stomach through the mouth.

A raised growth on the surface of the skin or other organ.

The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called trachea.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.