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.

Melanoma begins in skin cells called melanocytes (cells that color the skin). Diagnosing melanoma in children is difficult because melanoma looks similar to another type of skin tumor that starts in the melanocytes, which may need different treatment. New kinds of tests are being used to tell the difference between melanoma and these other tumors.

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, or a clinical trial of a monoclonal antibody. 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.

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