Surgical Management of Extra-Adrenal Abdominal Paragangliomas: Report of Three Cases and Review of the LiteraturePolistena, Andrea MD, PhD*; Cavallaro, Giuseppe MD, PhD*; Cotesta, Dario MD†; Petramala, Luigi MD†; Letizia, Claudio MD†; Basile, Ursula MD*; Filetti, Sepastiano MD†; Cavallaro, Antonino MD, FACS, MD*; De Toma, Giorgio MD*Author Information From the *Dipartimento di Chirurgia “Pietro Valdoni”, Policlinico Umberto I, Università “La Sapienza”, Roma, Italia; and †Dipartimento di Scienze Cliniche, Policlinico Umberto I, Università “La Sapienza”, Roma, Italia. Reprints: Giuseppe Cavallaro, MD, PhD, Dipartimento di Chirurgia “P. Valdoni,” Policlinico Umberto I, Viale del Policlinico, 00161–Rome, Italy. E-mail: [email protected] The Endocrinologist: March/April 2010 - Volume 20 - Issue 2 - pp 63-65 doi: 10.1097/TEN.0b013e3181d4bf9a Buy Metrics Abstract Introduction: The incidence of pheochromocytomas is about 1–2/100000 in the adult population. Nearly 10% have an extra-adrenal location or multiple locations and are defined as paragangliomas. They might be catecholamine-secreting as ordinary pheochromocytomas or clinically asymptomatic with compression symptoms. Patients and Methods: Three cases of abdominal paraganglioma with different clinical onset are reported. Discussion: Incidentally diagnosed asymptomatic retroperitoneal tumors with secondary hypertension suggest paraganglioma. Increased levels of catecholamine and extra-adrenal lesions identified at CT or MR imaging with positive MIBG scintigraphy support the diagnosis. Optimal care requires a multidisciplinary approach, accurate preoperative exact localization and adequate preoperative medical treatment to reduce perioperative cardiovascular complications. © 2010 Lippincott Williams & Wilkins, Inc.