Synchronous Multifocal Intraductal Tubulopapillary Neoplasm of the Pancreas Treated with Total Pancreatectomy: A Case Report.

INTRODUCTION

An intraductal tubulopapillary neoplasm (ITPN) is a rare pancreatic tumor characterized by scant mucin production. Even when associated with an invasive carcinoma component, its prognosis is generally considered more favorable than that of conventional pancreatic ductal adenocarcinoma. However, its pathogenesis and optimal treatment strategy remain unclear. Herein, we report a case of a synchronous multifocal ITPN treated with total pancreatectomy.

CASE PRESENTATION: A man in his 50s was referred to Gunma University Hospital for evaluation of pancreatic duct abnormalities after CT revealed a caliber change in the main pancreatic duct in the pancreatic body and distal pancreatic atrophy. Further evaluation demonstrated lesions associated with main pancreatic duct strictures in the pancreatic body and tail. A diagnosis of resectable pancreatic body and tail cancers was therefore established, and the patient received 2 courses of neoadjuvant chemotherapy (NAC) with gemcitabine plus S-1. Imaging after NAC revealed a new lesion in the pancreatic head. Endoscopic ultrasonography-guided fine-needle aspiration confirmed adenocarcinoma, and the patient was diagnosed with synchronous multifocal pancreatic cancers involving the pancreatic head, body, and tail. Total pancreatectomy was performed. Histopathological examination demonstrated 3 noncontiguous synchronous multifocal ITPNs, each with an independent invasive carcinoma component, in the pancreatic head, body, and tail. The postoperative course was uneventful, and the patient was discharged on POD 15. The patient required no adjuvant therapy and remained recurrence-free at 11 months postoperatively.

CONCLUSIONS

We describe an extremely rare case of a synchronous multifocal ITPN. The noncontiguous distribution observed in this case raises the possibility of multicentric development, although alternative mechanisms cannot be excluded. Further case accumulations and molecular analyses are required to clarify the pathogenesis of this disease and establish optimal treatment strategies.