Risk Factors And Causes For A Pheochromocytoma

Hereditary Paraganglioma Syndrome

Hereditary paraganglioma syndrome is part of a group of inherited cancers that includes pheochromocytomas. The syndrome is characterized by the presence of paragangliomas, which are tumors that develop from neuroendocrine tissues along the spine. Some of these tumors may secrete hormones. Doctors can perform a genetic test to diagnose hereditary paraganglioma syndrome. It is recommended that anyone with a pheochromocytoma and those with a family history of this disease or related conditions consider having the test. Individuals with a family history or genetic markers for hereditary paraganglioma syndrome should have full-body MRI scans every year or two beginning at ten years old to screen for this disease. Yearly blood tests should be performed to measure levels of catecholamines, and patients should also have a complete physical examination each year. During this exam, patients should be carefully assessed for high blood pressure. Patients should also avoid smoking and living in high altitude environments.

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Multiple Endocrine Neoplasia Type 2

Multiple endocrine neoplasia syndromes are uncommon genetic disorders where numerous endocrine glands in the body form tumors or grow larger abnormally without developing tumors. Multiple endocrine neoplasia type 2 (MEN type 2) specifically involves tumors or excessive growth in the thyroid gland, parathyroid glands, lips, tongue, gastrointestinal tract, and the adrenal glands. This tumor or overgrowth is caused by aberrant additional activation of the affected glands, which is itself a result of a malfunction or mutation of a tumor suppressor gene. This particular gene is located on chromosome 10, and it is called the RET or Rearranged during Transfection gene. Most often, changes in the glands due to MEN type 2 occur slowly over time, but the abnormal growth can take place in more than a single gland at a time. Almost all individuals with multiple endocrine neoplasia type 2 will develop medullary thyroid cancer, and about half of them will develop pheochromocytomas. When abnormal endocrine gland activation occurs in the adrenal glands of individuals with MEN type 2, a pheochromocytoma will likely develop.