Endocrine tumors arise in the glands of the endocrine system, which includes the thyroid, adrenal, pituitary and parathyroid glands. These glands produce various hormones that are essential for the body’s growth, metabolism and sexual development. Tumors arising from the endocrine system can be benign or malignant. Cancerous endocrine tumors are very rare in children, with thyroid cancers being the most common.
Endocrine tumors and cancers affect the following glands:
Thyroid: a butterfly-shaped gland in the neck that produces hormones involved in metabolism. Thyroid cancer is a rare disease in children under the age of 10 and is more common in adolescents ages 15 to 19. Fortunately, the prognosis is excellent for most cases of pediatric thyroid cancer, even if there is metastatic disease at diagnosis.
Pediatric thyroid tumors include benign thyroid nodules, papillary thyroid cancer, follicular thyroid cancer and medullary thyroid cancer.
Pituitary: a gland at the base of the brain which regulates growth, reproduction and other metabolic functions. Pituitary tumors are usually benign and can be either non-functioning or secrete too much hormone, which, for example, can cause gigantism or Cushing's disease.
Parathyroid: four tiny glands in the neck that produce hormones to regulate calcium and phosphorus levels in the blood. Tumors on these glands can cause overproduction of hormones (hyperparathyroidism).
Adrenal: two glands that sit atop the kidneys and produce hormones that control blood pressure and other functions. Adrenal tumors include adrenocortical tumors and pheochromocytoma.
Multiple endocrine neoplasias (MEN) are inherited conditions that can cause tumors of multiple glands.
von Hippel Lindau Syndrome (VHL) is a genetic condition that can cause blood vessel tumors of the central nervous system, kidney tumors, pancreas and kidney cysts and pheochromocytoma.
The diagnosis of endocrine tumors is usually made via an imaging procedure (usually ultrasound, CT scan, MRI, and/or nuclear medicine tests) as well as a thorough evaluation of hormone levels. In cases of a thyroid tumor, a fine needle aspiration (biopsy) is often utilized to render the correct diagnosis.
Benign endocrine tumors are treated with medications, observation and/or surgery. Treatment of an endocrine cancer varies according to the type of tumor, but typically involves surgery, medications (including chemotherapy in advanced cancers), and radioactive iodine in the case of certain thyroid cancers.
The treatment of endocrine tumors at MD Anderson is a coordinated effort between specialists who are experts in the management of children with these tumors, including surgical experts, pediatric oncologists, nuclear medicine physicians, radiologists and genetic counselors.