Disease Information

Solid Tumor: Endocrine Tumors

Alternate Names: None

Definition

Cancers of the endocrine glands are very uncommon in children. Endocrine tumors affect endocrine organs, which are organs that secrete hormones that regulate many of the body’s functions.

• About 40-45 percent of childhood endocrine tumors arise from the gonads (e.g. germ cell, testicular and ovarian tumors).
• About 30 percent arise from the thyroid gland, a gland located in the neck that manufactures hormones that affect heart rate, body temperature and energy level, and controls the level of calcium in the blood.
• About 20 percent originate in the pituitary, a small organ about the size of a pea in the center of the brain just above the back of the nose. The pituitary gland makes hormones that affect the growth and the functions of other glands in the body.

The other, more rare, endocrine cancers affect:

• The parathyroids, which are four small endocrine glands located adjacent to or embedded in the thyroid. They produce a hormone concerned with calcium metabolism.
• The adrenal cortex and medulla. The adrenal cortex is the outside layer of the adrenal gland. Cancer of the adrenal cortex is also called adrenocortical carcinoma. There are two adrenal glands, one above each kidney in the back of the upper abdomen. The inside layer of the adrenal gland is called the adrenal medulla. Cancer that starts in the adrenal medulla is called pheochromocytoma. The cells in the adrenal cortex make hormones that help the body work properly. When cells in the adrenal cortex become cancerous, they may make too much of one or more hormones, which can cause symptoms such as high blood pressure, weakening of the bones or diabetes. If male or female hormones are affected, the body may go through changes such as a deepening of the voice, growing hair on the face, premature development of the sex organs or swelling of the breasts.
• The pancreas, which lies behind the stomach, inside a loop formed by part of the small intestine. It produces digestive juices that help break down food and hormones (such as insulin) that regulate how the body stores and uses food.

Most endocrine tumors of childhood are benign or low-grade malignancies. A small percentage of gonadal and germ cell tumors, thyroid cancers, and adrenocortical tumors are high-grade malignancies. Malignant carcinomas of the parathyroids, the adrenal medulla, and the pancreas are very rare in children. Many of these tumors produce hormones. Measuring the blood levels of these hormones can be useful in the diagnosis and monitoring the effects of treatment.

Incidence

Endocrine tumors represent about 4-5 percent of all cancers seen in children.

Influencing Factors

• Some endocrine tumors are hereditary. Medullary carcinomas of the thyroid and pheochromocytomas can occur in families.
• Multiple endocrine neoplasia (MEN) syndromes are familial disorders in which cancer arises simultaneously in more than one endocrine gland.

Survival Rates

• Prognosis for patients with pituitary and thyroid tumors and adrenal adenomas is generally good.
• The prognosis for adrenal carcinoma is generally poor, with a mean survival of about 18 months.
• The outcome for patients with the MEN syndrome is generally good, provided adequate treatment can be obtained for each type of tumor.
• The prognosis for patients with ovarian or testicular germ cell tumor is excellent with most patients achieving disease-free long-term survival.

Treatment Strategies

• Surgery is generally the treatment of choice for most endocrine tumors.
• Chemotherapy is frequently used.  It is most effective in germ cell tumors of the ovary and testes.
• Some tumors are resistant to radiation therapy. Even in these cases, radiation therapy after the tumor has been removed may decrease the chance of recurrence. It is also helpful in halting the spread of the cancer in some cases.
• Hormone replacement therapy is frequently a part of treatment.

Current Research

• For adrenocortical carcinoma (ACC), St. Jude is currently participating in an international study in which researchers are investigating a new surgery technique that is enhanced by chemotherapy treatment.
• Researchers are growing tumor cells in cultures to study new drugs for treatment.
• St. Jude participated in the discovery of a new gene mutation in some patients with ACC. An epidemiological genetic study is being held to understand more about this gene mutation and its role to the incidence, early diagnosis and treatment of disease. Epidemiology is a branch of medical science that deals with the incidence, distribution, and control of disease in a population.
• For germ cell tumors, current efforts are focused on reducing the amount of chemotherapy while maintaining the excellent cure rates. Because these tumors are so rare, clinical trials are done in national, multi-institutional collaborations.

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