Abstract
A 59-year-old man with an extremely rare case of having both hypogammaglobulinemia and type 1 diabetes mellitus, was diagnosed with an intraductal papillary mucinous neoplasm (IPMN) at the age of 53 years. The IPMN had been gradually developing for 6 years after diagnosis ; the largest cyst grew to over 30 mm with an enhancing cyst wall, and the main pancreatic duct was dilated to over 10 mm. He underwent total pancreatectomy for the IPMN. Since we assumed that the risk of surgical complication due to his comorbidity was more significant, we performed total pancreatectomy. The pathological diagnosis based on the resected specimen was intraductal papillary mucinous carcinoma, TS2 (33 mm), pTisN0. After surgery, the patient underwent the usual protocol except appropriate immunoglobulin supplementation. To the best of our knowledge, there have been no reports of cases of total pancreatectomy in patients having both hypogammaglobulinemia and type 1 diabetes mellitus ; thus, our present report details this extremely rare case.
