Concomitant Case of Intraductal Papillary Mucinous Neoplasm of the Pancreas and Functioning Pancreatic Neuroendocrine Tumor (Vasoactive Intestinal Polypeptide-Producing Tumor): First Report

To the Editor: I ntraductal papillary mucinous neoplasm (IPMN) of the pancreas co-occurs rarely with a pancreatic neuroendocrine tumor (PNET), and only 13 cases were reported up to 2009. It is unusual that all of these cases involved nonfunctioning PNETs and that no cases of IPMN with a functioning PNET have been reported to date. Here, we report the first case of IPMN concomitant with a functioning PNET that produced vasoactive intestinal polypeptide (VIP).

[1]  A. Maitra,et al.  Whole-exome sequencing of neoplastic cysts of the pancreas reveals recurrent mutations in components of ubiquitin-dependent pathways , 2011, Proceedings of the National Academy of Sciences.

[2]  Michael A. Choti,et al.  DAXX/ATRX, MEN1, and mTOR Pathway Genes Are Frequently Altered in Pancreatic Neuroendocrine Tumors , 2011, Science.

[3]  F. Bosman,et al.  WHO Classification of Tumours of the Digestive System , 2010 .

[4]  G. Costamagna,et al.  Concomitant intraductal papillary mucinous neoplasm and pancreatic endocrine tumour: Report of two cases and review of the literature. , 2009, Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver.

[5]  J. Cameron,et al.  Pancreatic VIPomas: Subject Review and One Institutional Experience , 2008, Journal of Gastrointestinal Surgery.

[6]  P. Chow,et al.  Clinicopathological Features of Patients with Concomitant Intraductal Papillary Mucinous Neoplasm of the Pancreas and Pancreatic Endocrine Neoplasm , 2007, Pancreatology.

[7]  A. Sauvanet,et al.  Endocrine Tumor and Intraductal Papillary Mucinous Neoplasm of the Pancreas: A Fortuitous Association? , 2005, Pancreas.

[8]  Y. Morioka,et al.  Clinical pathology of endocrine tumors of the pancreas , 1991, Digestive Diseases and Sciences.

[9]  L. Kvols,et al.  Consensus report on the use of somatostatin analogs for the management of neuroendocrine tumors of the gastroenteropancreatic system. , 2004, Annals of oncology : official journal of the European Society for Medical Oncology.

[10]  J. K. Martin,et al.  Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. , 1998, Surgery.