Elliot Mitmaker

By Tanya Castelino and Elliot Mitmaker

Pheochromocytomas are rare tumours of the adrenal gland that secrete catecholamines. The classical presentation of these tumours consists of a clinical triad of headaches, palpitations and diaphoresis. This clinical presentation should not be confused with the potentially fatal presentation of pheochromocytoma crisis, which may include severe haemodynamic instability and collapse, multi‐organ failure, hyperthermia and encephalopathy. When patients present in profound shock, supportive care and treatment are initiated. Patients presenting with pheochromocytoma crisis have an underlying adrenal tumour, but the clinical manifestations of this life‐threatening condition can mimic other entities. Once diagnosis is made, previous anecdotal evidence has shown that pheochromocytoma crisis is a surgical emergency. However, retrospective study of a larger sample of patients presenting with pheochromocytoma crisis suggests that medical management in the acute setting is appropriate and safe. The ultimate treatment is indeed surgical; however, there is no clear recommendation for the acute management of pheochromocytoma crisis. This chapter will focus on the medical and surgical management of potentially life‐threatening pheochromocytoma crisis. An in‐depth review of the clinical presentation, pathophysiology, causes and treatments of pheochromocytoma crisis will be provided, including the controversial areas surrounding decision‐making and timing for adrenalectomy.

Part of the book: Clinical Management of Adrenal Tumors