Excision of bilateral pheochromocytomas followed by staged resection of neuroendocrine carcinoma of the pancreas

Abstract Pheochromocytoma (PCC) and Pancreatic Neuroendocrine tumor (PNET) occurring together have been considered as a spectrum of Von Hippel Lindau (VHL) Syndrome and carries a grave prognosis. Considering the rarity of the disease, there is a wide range of presentations, investigations and management options described in literature. We describe one such case that was managed by staged excision of bilateral pheochromocytoma followed by pancreatic neuroendocrine tumor with a five years follow up and also discuss in detail our review of literature about this condition.