Gut Liver.  2012 Jul;6(3):287-294.

The Current Strategy for Managing Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1

• Niina Y
• Fujimori N
• Nakamura T
• Igarashi H
• Oono T
• Nakamura K
• Kato M
• Jensen RT
• Ito T
• Takayanagi R

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is an inherited autosomal dominant disease presenting with pancreatic neuroendocrine tumors (pNETs), parathyroid tumors, or pituitary tumors. Using the PubMed database, we reviewed the literature on information regarding the proper diagnosis and treatment of MEN1-associated pNET. Many cases of MEN1-associated pNET are functioning pNETs. Gastrinomas and insulinomas tend to occur frequently in the duodenum and pancreas, respectively. In addition to diagnostic imaging, the selective arterial secretagogue injection test (SASI test) is useful for localizing functioning pNET. The standard treatment is surgical resection. However, in the case of a functioning pNET, the tumor should first be accurately located using the SASI test before an appropriate surgical method is selected. In cases of a MEN1-associated non-functioning pNET that exceeds 2 cm in diameter, the incidence of distant metastasis is significantly increased, and surgery is recommended. In cases of unresectable pNET, a somatostatin analog has been shown to demonstrate antitumor effects and is considered to be a promising treatment. In addition, molecular-targeted drugs have recently been found to be effective in phase III clinical trials.

Keyword

Multiple endocrine neoplasia type 1; Pancreatic neuroendocrine tumor; Multiple tumors; Selective arterial secretagogue injection test

MeSH Terms

Diagnostic Imaging
Duodenum
Gastrinoma
Incidence
Insulinoma
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive
Neuroendocrine Tumors
Pancreas
Pituitary Neoplasms
Somatostatin
Somatostatin