[Pheochromocytoma and paraganglioma: basics for the general practitioner]. / Phéochromocytome et paragangliome: que doit retenir le praticien?

ABSTRACT

Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare neuroendocrine tumors secreting catecholamines in most cases. The clinic can be very variable. Morbidity and mortality PHEO and PGL are primarily cardiovascular and haves catecholamine-mediated origin. The PHEO and PGL can sign in with a syndromic association with multiple tumors and genetic counseling is necessary in search of a germline mutation. The first step includes a diagnostic assay of metanephrine and normetanephrines. In case of positive biology a CT or MRI imaging will be needed to locate the tumor. Treatment with alpha-blocker before surgery reduces the perioperative risk. A long term follow up is recommended to detect recurrence.