Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas.
Introduction Somatostatinomas of the ampulla are rare neuroendocrine tumours with limited studies in the literature. These are often associated with familial genetic predisposition e.g. NF 1 and Von Hippel-Lindau Syndrome. Histology commonly shows classical features such as Psammoma bodies. The classical presentation with inhibitory syndrome is rare, but ampullary mass effects can cause an earlier presentation with potentially better outcomes with earlier intervention and treatment. Case series We report three cases of ampullary somatostatisnomas: one sporadic and two familial, associated with neurofibromatosis type 1. The first patient presented with pruritus, the second with recurrent pancreatitis and the third, with elevated CA19-9 levels. Various preoperative localisation techniques were employed and one had an attempted endoscopic resection yielding involved margins. All patients underwent pancreaticoduodenectomy, of which one was laparoscopic assisted. The median size of the tumour was 10 mm and one patient had nodal involvement. All 3 patients have remained disease free at most recent follow up ranging from 1.5 to 11 years. Discussion Ampullary somatostatinomas can present early with mass related effects while inhibitory syndrome is rare. Early detection and intervention in ampullary somatostatinoma may contribute to better outcomes than pancreatic somatostatinomas. Long-term survival is achievable through pancreaticoduodenectomy for resectable ampullary somatostatinoma and laparoscopic approach is a feasible and viable option. Received December 25th, 2015 Accepted February 25th, 2016