Unusual Cancers of Childhood Treatment (PDQ®)

General Information

Different types of rare childhood tumors are discussed in this summary. Because these tumors are rare in children, there may not be much information about the best treatment. Sometimes the treatment of these tumors in children is the same treatment used in adults. See the NCI Web site for information about adult cancer and its treatment.


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.

Cancer of the Nose and Throat

Cancers that start in the lining of the nasal cavity and throat are called nasopharyngeal cancers. This type of cancer is very uncommon in children younger than 10 years, and occurs slightly more often in older children and teenagers aged 10 to 19 years.

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

Esthesioneuroblastoma

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.

Thyroid Tumors

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. See the Multiple Endocrine Neoplasia Syndromes (MEN) and Carey Complex section of this summary for more information.

Thyroid cancers occur less often in children and adolescents younger than 15 years, and more often in those aged 15 to 19 years. Most thyroid cancers occur 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. (See the PDQ summary on adult Thyroid Cancer Treatment for more information.)

Oral (Mouth) Cancers

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. Mucoepidermoid carcinomas are rare and may be cured with surgery alone. Treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy. (See the PDQ summaries on adult Oropharyngeal Cancer Treatment and Lip and Oral Cavity Cancer Treatment for more information).

Salivary Gland Tumors

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

Laryngeal Cancer and Papillomatosis

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 children and is usually treated with chemotherapy and radiation therapy instead of surgery. Squamous cell carcinoma of the larynx should be managed with surgery and radiation therapy. 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) and is treated with laser surgery. 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 that rarely extend into the lung. Cancerous changes may develop over time in the larynx and lung. (See the PDQ summary on adult Laryngeal Cancer Treatment for more information.)

Respiratory Tract Cancer with Chromosome 15 Changes

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 cancers are rare in patients younger than 15 years of age.

Breast Cancer

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 for children and adolescents with breast cancer include surgery and radiation therapy. 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).(See the PDQ summary on adult Breast Cancer Treatment for more information.)

Bronchial Tumors

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 Blastoma

Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs. Changes in a specific chromosome may be found in pleuropulmonary blastoma. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Part of the DNA in chromosome 8 is often abnormal in these tumors. 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. There are no standard treatment options for pleuropulmonary blastoma. Early-stage tumors may be treated with surgery alone, or surgery with adjuvant chemotherapy to help prevent the tumor from coming back. Late-stage tumors may be treated with surgery followed by chemotherapy. 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. Pleuropulmonary blastoma may also occur in siblings of patients.

Esophageal Tumors

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

Thymomas and Thymic Carcinoma

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 or neuromyotonia (a rare nerve disorder that causes constant muscle stiffness and cramping), 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. (See the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.)

Tumors of the Heart

The most common tumors that begin in the heart are benign. In children, these include rhabdomyomas (tumors of muscle tissue) and fibromas (tumors of fibrous tissue). Myxomas are rare, benign tumors which may be part of an inherited syndrome called Carney complex (see Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex section below for more information). Other benign tumors include histiocytoid cardiomyopathy tumors, teratomas, hemangiomas, and neurofibromas. Malignant tumors that begin in the heart are rare, but may include malignant teratomas, rhabdomyosarcomas, and chondrosarcomas.

Patients may have no symptoms for long periods of time. Symptoms include abnormal heart rhythm, enlargement of the heart, fluid in the sac around the heart, and congestive heart failure. Sudden death with no early symptoms is possible. Successful treatment may require surgery (which may include transplantation) and chemotherapy. Benign tumors of heart muscle (rhabdomyomas) usually shrink and go away on their own.

Mesothelioma

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. (See the PDQ summary on adult Malignant Mesothelioma Treatment 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. (For more information, see the Renal Cell Cancer section in the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors.)

Cancer of the Adrenal Cortex

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

Cancer of the Stomach

Cancer that begins in the stomach is rare in children. 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. (See the PDQ summary on adult Gastric Cancer Treatment for more information.)

Cancer of the Pancreas

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

Colorectal Cancer

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. (See the PDQ summaries on adult Colon and Rectal Cancer Treatment for more information.)

Carcinoid Tumors

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

Gastrointestinal Stromal Tumors

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 and is rare in children. GIST is more common in girls, and usually appears in patients over the age of 10 years. Most children with GIST have tumors in the stomach and develop anemia caused by gastrointestinal bleeding. A small number of children with GIST are found to have one of two rare genetic disorders, Carney triad or Carney Stratakis syndrome.

GIST in children is not the same as the disease in adults. Patients should be seen at centers that specialize in the treatment of GIST and all tumors should be tested for genetic changes. A small number of children have tumors with genetic changes similar to those found in adult patients. These children are treated with a type of drug called a tyrosine kinase inhibitor that blocks these changes.

Treatment for most children whose tumors do not show these genetic changes includes surgery to remove the tumor when possible. If the disease comes back in the same place or cannot be removed but is not causing symptoms, watchful waiting may be recommended. If the disease progresses or causes symptoms, tyrosine kinase inhibitor therapy may be an option. However, there is not enough evidence to recommend tyrosine kinase inhibitor therapy as standard adjuvant treatment in children.


Genital/Urinary Tumors

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

Bladder Cancer

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 Bladder Cancer Treatment drugs given for other childhood tumors or leukemia. (See the PDQ summary on adult for more information.)

Testicular Cancer

Testicular tumors are very rare in young boys. There are 2 types of testicular tumors, germ cell and non-germ cell tumors. Germ cell tumors begin in cells that give rise to sperm or eggs, and can be either benign or malignant. Testicular germ cell tumors include benign teratomas (the most common testicular tumors in boys), and malignant nonseminomas. (See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information). Non-germ cell tumors, such as stromal tumors, are very rare in boys, and are usually benign. Testicular tumors in young boys may be treated with surgery, but there are few studies in children.

Ovarian Cancer

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. Sertoli-Leydig cell tumors of the ovary are rare in young girls and may cause development of male sex traits or early signs of puberty. Small cell carcinomas of the ovary are very rare and fast-growing tumors that may cause higher than normal levels of calcium in the blood. Small cell carcinomas may be treated with surgery and chemotherapy, but there are few studies in children. (See the PDQ summaries on Childhood Extracranial Germ Cell Tumors Treatment, Ovarian Epithelial Cancer Treatment, Ovarian Germ Cell Tumors Treatment, or Ovarian Low Malignant Potential Tumors Treatment for more information.)

Carcinoma of the Cervix and Vagina

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 (MEN) and Carney Complex

Multiple endocrine neoplasia syndromes or MEN are familial disorders that cause abnormal 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.

These disorders, called MEN 1, MEN 2A, MEN 2B, and familial medullary carcinoma of the thyroid (FMTC), are caused by abnormal genes. Patients and family members at risk for these syndromes should have both genetic testing and physical examinations.

MEN 1 (Werner syndrome) may involve tumors of the pituitary and parathyroid glands, adrenal, gastric, and pancreatic structures.

MEN 2A (Sipple syndrome) is associated with medullary thyroid carcinoma (cancer that forms in the cells of the thyroid that make the hormone calcitonin) and parathyroid hyperplasia, as well as pheochromocytoma (a usually benign tumor that forms in the center of the adrenal gland, causing it to make too much adrenaline). MEN 2B is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma, mucosal neuromas, and ganglioneuromas.

Patients with the MEN 2A syndrome usually have surgery to remove the thyroid by the age of 5 years or at the time genetic changes are found 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.

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.

Familial medullary thyroid carcinoma (FMTC) is an inherited form of medullary thyroid cancer that occurs without pheochromocytoma or parathyroid adenoma/hyperplasia.

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 Carney complex is an inherited syndrome that causes myxomas (benign tumors) of the heart and skin, lentigines (brown sun spots on the skin), blue nevi (benign moles), Cushing syndrome, and endocrine and non-endocrine tumors. For patients with the Carney complex, prognosis depends on how often heart, skin, or other tumors recur.

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.

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.

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

Melanoma is 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 occurs less often in children younger than 10 years, and occurs more often in older children and adolescents aged 10 to 19 years. The rate of melanoma in children has increased in recent decades. Higher levels of ultraviolet light from the sun increases the risk of this cancer.

Conditions that increase the risk of melanoma in children and adolescents include giant melanocytic nevi (large congenital black spots which may cover the trunk and thigh), xeroderma pigmentosum (a rare genetic condition marked by an extreme sensitivity to sunlight), certain disorders of the immune system, and Werner syndrome. Traits in all age groups that increase the risk of melanoma include being exposed to ultraviolet radiation, having red hair, blue eyes, or freckling, poor tanning, and family history of melanoma. Melanoma in children is similar in many ways to the disease in adults, although the thickness of lesions in children does not seem to predict how likely it is that the melanoma will be cured.

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. Information about ongoing clinical trials for melanoma in children aged 10 years or older is available from the NCI Web site.

Chordoma

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, chordomas develop more often in the base of the skull, making them difficult to completely remove with surgery. 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 of Unknown Primary Site

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 age and 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

  • Childhood Cancers

  • CureSearch - National Childhood Cancer Foundation Children’s Oncology Group

  • 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: Questions and Answers

  • Understanding Cancer Series: Cancer

  • Staging: Questions and Answers

  • Coping with Cancer: Supportive and Palliative Care

  • Cancer Library

  • Information for Survivors/Caregivers/Advocates


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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 (08/13/2009)

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. 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).


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