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World J Gastroenterol. Sep 28, 2010; 16(36): 4519-4525
Published online Sep 28, 2010. doi: 10.3748/wjg.v16.i36.4519
Recent standardization of treatment strategy for pancreatic neuroendocrine tumors
Masayuki Imamura
Masayuki Imamura, Department of Surgey, Kansai Denryoku Hospital, 2-1-7, Fukushima, Fukushima-Ku, Osaka 553-0003, Japan
Author contributions: Imamura M contributed wholly to this paper.
Correspondence to: Masayuki Imamura, MD, FACS, Department of Surgey, Kansai Denryoku Hospital, 2-1-7, Fukushima, Fukushima-Ku, Osaka 553-0003, Japan. imamura.masayuki@c4.kepco.co.jp
Telephone: +81-6-64585821 Fax: +81-6-64586994
Received: February 2, 2010
Revised: March 3, 2010
Accepted: March 10, 2010
Published online: September 28, 2010
Abstract

Recent advances in localization techniques, such as the selective arterial secretagogue injection test (SASI test) and somatostatin receptor scintigraphy have promoted curative resection surgery for patients with pancreatic neuroendocrine tumors (PNET). For patients with sporadic functioning PNET, curative resection surgery has been established by localization with the SASI test using secretin or calcium. For curative resection of functioning PNET associated with multiple endocrine neoplasia type 1 (MEN 1) which are usually multiple and sometimes numerous, resection surgery of the pancreas and/or the duodenum has to be performed based on localization by the SASI test. As resection surgery of PNET has increased, several important pathological features of PNET have been revealed. For example, in patients with Zollinger-Ellison syndrome (ZES), duodenal gastrinoma has been detected more frequently than pancreatic gastrinoma, and in patients with MEN 1 and ZES, gastrinomas have been located mostly in the duodenum, and pancreatic gastrinoma has been found to co-exist in 13% of patients. Nonfunctioning PNET in patients with MEN 1 becomes metastatic to the liver when it is more than 1 cm in diameter and should be resected after careful observation. The most important prognostic factor in patients with PNET is the development of hepatic metastases. The treatment strategy for hepatic metastases of PNET has not been established and aggressive resection with chemotherapy and trans-arterial chemoembolization have been performed with significant benefit. The usefulness of octreotide treatment and other molecular targeting agents are currently being assessed.

Keywords: Gastrinoma, Glucagonoma, Insulinoma, Multiple endocrine neoplasia type 1, Octreotide, Pancreas preserving total duodenectomy, Pancreatic neuroendocrine tumors, Selective arterial secretagogue injection test, Somatostatin receptor scintigraphy