Current management of patients with pheochromocytoma

Abstract

Pheochromocytomas are neuroendocrine catecholamine secreting tumors that arise from chromaffin cells of the adrenal medulla or rarely from the sympathetic and parasympathetic ganglions. They have a wide spectrum of pathological and clinical manifestations. Headache, sweating and palpitations, combined with persistent or paroxysmal hypertension, are described as the most typical and highly specific, but far less sensitive symptoms. Due to better availability and accessibility of imaging procedures, an increasing proportion of pheochromocytomas are discovered incidentally upon abdominal imaging, stressing the importance of appropriate screening in all patients with adrenal incidentaloma. The diagnosis of pheochromocytoma is confirmed by measuring catecholamines and metanephrines in a 24-hour urine collection. Biochemical confirmation is followed by abdominal CT (or MR) imaging and 123I–metaiodobenzylguanidine radioisotope scanning to locate the tumor. Imaging phenotype of pheochromocytoma is well defined and should sometimes guide the management in asymptomatic patients with normal biochemical tests. A significant progress has been made in understanding the genetic determinism of these tumors. Genetic testing should be offered to selected patients with appropriate phenotype. The only curative treatment is surgical. A laparoscopic approach after careful preoperative pharmacologic preparation with long-term alpha-adrenergic blockade is currently regarded as safe with very few complications. It is crucial to maintain a high index of suspicion for pheochromocytoma, as the tumor is highly curable when recognized and potentially lethal when missed. The literature review was complemented by retrospective analysis of pheochromocytoma patients treated at our institution from 2006 to 2010. Our results are comparable to results published in recent papers and in accordance with current guidelines.