Subclinical Pheochromocytoma and Paraganglioma in an Elderly Patient: A Case Report

Pheochromocytomas and paragangiomas are a class of neuroendocrine tumors with a widely variable clinical presentation ranging from paroxysmal episodes of critically elevated blood pressure to no clinical symptoms. Genetic predisposition to pheochromocytomas is well documented. Initial workup entails biochemical evaluation of urine catecholamine metabolites, intravenous contrast enhanced anatomic imaging for adrenal gland evaluation, and functional imaging with methy-liodobenzylguanidine (MIBG) whole-body scintigraphy for localization of extra-adrenal disease. We present an atypical case of concomitant pheochromocytoma and retroperitoneal paraganglioma in an elderly 78-year-old female found to have incidental bilateral adrenal and retroperitoneal masses on abdominal CT since 3 years. Subsequent biochemical workup was consistent with pheochromocytoma. The patient denied any headache, sweating, or palpitations or other suggestive symptoms. She was then lost to followup for 3 years until she presented to our institution for ischemic leg pain warranting urgent vascular surgery pending surgical resection of her pheochromocytoma. Following repeat workup and surgical resection, histopathology confirmed right adrenal pheochromocytoma and retroperitoneal paraganglioma. This case is highly atypical of pheochromocytoma given the old age at presentation, subclinical course, and associated paraganglioma, arousing suspicion for familial disease.