Pheochromocytoma: presentation, diagnosis and treatment

Abstract

Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion. Typical clinical manifestations are sustained or paroxysmal hypertension, severe headaches, palpitations and sweating resulting from hormone excess. However, their presentation is highly variable and can mimic many other diseases. If remaining unrecognized or untreated, they can be a life-threatening condition. Therefore, the most important message of this review is to think of them. The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines. They are localized by a computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen; complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography are available. Because approximately one out of four pheochromocytomas turn out to be hereditary entities, screening for genetic alterations is important. Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative. In malignant pheochromocytomas, radiotherapy and chemotherapy are palliative treatment options. This review provides an update on identification and management of pheochromocytomas, emphasizing current developments in diagnosis, including genetic testing, pathophysiology and treatment of pheochromocytomas.

Malignant giant pheochromocytoma: a case report and review of the literature

“…10 For malignant tumours, due to their low incidence, only isolated cases are published rather than large series, so it is difficult to determine the outcomes.…”

“…Hormonal blocking with 131-I-MIBG could be useful in residual or irresectable disease. 10 Follow-up is also important and, with time, we can determine the malignant tumoural behaviour.…”

Perioperative Management of Adrenalectomy and Inferior Vena Cava Reconstruction in a Patient With a Large, Malignant Pheochromocytoma With Vena Caval Extension

“…45 Although a mortality rate of close to zero has been reported in all recent series of adrenalectomy for pheochromocytoma, irrespective of preoperative care, 45 this improved prognosis currently is attributed (1) to the widespread preoperative administration of vasodilating drugs, even to patients with normal arterial pressure in some series, and (2) to the administration of large fluid volumes, even to patients with normal or borderline elevation of catecholamine levels in other series. 1 None of these practices was evidence based. 45 Are Patients With Pheochromocytoma Hypovolemic?…”

“…Pheochromocytoma is a rare neuroendocrine tumor that arises from chromaffin cells of the adrenal medulla or extraadrenal paraganglia that produces and releases catecholamines and their metabolites. 1 The only cure for pheochromocytoma is surgical resection, with most recent studies demonstrating minimal or zero perioperative mortality, which commonly is attributed to improved preoperative preparation. 2,3 There are case reports demonstrating that large pheochromocytomas have the potential to invade into the IVC and result in significant IVC thrombus formation.…”

An Extra-adrenal Pheochromocytoma Presenting as Malignant Hypertension-A Report of two Cases

“…The symptoms of a pheochromocytoma can further be mimicked by hyperthyroidism, panic attacks, hypoglycaemia and alcohol withdrawal symptoms. The sudden cessation of a clonidine of the beta blocker therapy may also cause similar symptoms [7].…”