Management and Treatment of Pheochromocytomas and Paragangliomas

Abstract

Pheochromocytomas and paragangliomas are rare neural crest-derived tumors of sympathetic (generally catecholamine producing) or parasympathetic (rarely catecholamine producing) origin. Patients affected by these tumors present with a variable clinical picture, often making diagnosis troublesome. Surgery is the treatment of choice, but requires appropriate medical management before, during, and after tumor resection. Appropriate follow-up of patients is particularly important to identify recurrences, remaining disease, or developing malignancy. Currently, however, no firm guidelines exist about what form follow-up should take. There is also a general lack of prospective studies establishing the best approaches for management and treatment of the tumor. Choice of the many available different therapeutic options instead usually depends on institutional experience and clinical setting, which may vary for different groups of patients. At the First International Symposium on Pheochromocytoma (ISP2005), held in Bethesda in October 2005, a panel of experts addressed and discussed the many therapeutic options and problems associated with management and treatment of patients with pheochromocytoma, reporting their personal experience and sharing their opinions with those of patient representatives. The aim of this special Discussion Session was to reconcile differences of opinion and reach agreement about appropriate management and therapeutic options. This article summarizes the discussion and the recommendations derived from that session.