Phéochromocytomes et paragangliomes abdominaux

Pheochromocytomas and paragangliomas are tumors derived from the sympathetic and parasympathetic nervous system. The parasympathetic-associated paragangliomas arising in the neck are usually non-functioning and are rarely seen by general and visceral surgeons. The sympathetic-associated pheochromocytomas and paragangliomas are usually functioning and most often arise in the abdomen. Because they harbor very specific characteristics (hypersecretion of catecholamines, familial origin in up to 30% of them, multiple locations, etc.), their perioperative management needs to be known by surgeons taking care of these patients in order to avoid operative disasters. Surgery can lead to peroperative hemodynamic modifications and sometimes catecholamine storms even in normotensive patients with pheochromocytomas and paragangliomas. This emphasizes the need to exclude pheochromocytomas before any adrenal surgery as well as to prepare all patients with pheochromocytomas and paragangliomas preoperatively. We review in this paper the pathophysiology and current perioperative management of patients with apparently sporadic pheochromocytomas and paragangliomas.