Phaeochromocytoma

What it is

Pheochromocytomas are rare tumors that produce, store, and secrete catecholamines. They are found in the adrenal medulla, with 10% extra-adrenal. As a catecholamine-secreting adrenal tumor they cause episodic hypertension, and they sit among the causes of secondary hypertension, appearing as the catecholamines entry in the list of causes of secondary and refractory hypertension.

Causes and risk

Further workup is prompted by resistant hypertension, paroxysmal hypertension, episodes of headaches, palpitations and diaphoresis, a family history of endocrine tumors, and cutaneous features suggestive of NF type 1. An adrenal incidentaloma is another prompt, as is a genetic condition such as Von-Hippel-Lindau or MEN 2A or 2B. Secondary hypertension in general is evaluated when the patient is under 40 or the hypertension is resistant.

The multiple endocrine neoplasms are all autosomal dominant, and when one is seen the others are thought of, especially in a young patient. Pheochromocytoma stands in two of the three rows set out. MEN 2A, the PMP row, is parathyroid, medullary thyroid cancer and pheochromocytoma; MEN 2B, the MMP row, is marfanoid habitus with mucosal neuromas, medullary thyroid cancer and pheochromocytoma. The third row, MEN 1 or PPP, is parathyroid, pituitary adenoma and pancreatic tumors secreting insulin, VIP or gastrin, and it holds no pheochromocytoma.