A case of mistaken identity: giant cystic pheochromocytoma

Abstract

A 39-year-old Marshall Islands woman was referred for evaluation of an abdominal mass. Medical history was significant only for pulmonary tuberculosis and scrofula. The patient denied a personal or family history of pancreatic or endocrine disease. Physical examination revealed normal vital signs and a 12-cm mildly tender left upper quadrant mass. A computed tomography scan revealed an 11-cm cystic mass contiguous with the distal pancreas and closely associated with the hilum of the spleen and the left kidney. Based on these findings and the patient's history, a cystic neoplasm of the pancreas was suspected, and she was subsequently taken to the operating room for exploration. Intraoperatively, the patient became markedly hypertensive with manipulation of the mass, requiring intravenous nitroprusside. Histopathological evaluation confirmed the diagnosis of cystic pheochromocytoma (PCC). The patient's postoperative course was uncomplicated. Cystic PCCs may not present with the classic prodromal symptoms associated with solid PCCs. This case represents the complex and unsuspected presentation of an extremely rare functional cystic neoplasm. A high index of suspicion for cystic PCC is necessary when confronted with cystic lesions in the vicinity of the adrenal glands. Failure to recognize cystic PCC before resection may lead to uncontrollable hypertension in the operating room, with potentially serious consequences.