Surgical management of extra-adrenal abdominal paragangliomas: Report of three cases and review of the literature

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 catecholaminesecreting 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.