Paraganglioma

A paraganglioma is a rare tumor that develops from neuroendocrine cells found in clusters throughout the body, most often in the head, neck, chest or abdomen; when the same kind of tumor arises in the adrenal gland it is instead called a pheochromocytoma. Paragangliomas are uncommon, with an estimated overall incidence of about one in three hundred thousand, and there is no reliable test to tell a benign tumor from a malignant one at the time it is found, so long-term monitoring is recommended for everyone diagnosed with one. Roughly three-quarters of cases occur without any known family history, while the remaining quarter are hereditary and linked to mutations in one of several genes, including the SDH family of genes as well as VHL, NF1, TMEM127 and MAX, reflecting a range of distinct genetic pathways that can give rise to the same kind of tumor.