Pheochromocytoma and Paraganglioma

Paragangliomas are rare tumors that form in nerve tissue in the adrenal glands and near certain blood vessels and nerves in the head and neck, and in other parts of the body. Paragangliomas that form in the adrenal glands are called pheochromocytomas, while those that form outside the adrenal glands are called extra-adrenal paragangliomas or paragangliomas. Pheochromocytomas and paragangliomas may be benign or malignant.

The adrenal glands make important hormones called catecholamines that help control heart rate, blood pressure, and blood sugar. Some paragangliomas make extra catecholamines, the hormones called adrenaline and noradrenaline that may cause signs or symptoms of disease.

Certain inherited syndromes or gene changes increase the risk of pheochromocytoma or paraganglioma, including multiple endocrine neoplasia 2 (MEN 2) syndrome, von Hippel-Lindau (VHL) syndrome, and neurofibromatosis type 1 (NF1).

Pheochromocytoma and Paraganglioma Treatment (PDQ®)

Source: National Cancer Institute