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( Yusuke Niina ),( Nao Fujimori ),( Taichi Nakamura ),( Hisato Igarashi ),( Takamasa Oono ),( Kazuhiko Nakamura ),( Masaki Kato ),( Robert T. Jensen ),( Tetsuhide Ito ),( Ryoichi Takayanagi ) The Editorial Office of Gut and Liver 2012 Gut and Liver Vol.6 No.3
Multiple endocrine neoplasia type 1 (MEN1) is an inherited autosomal dominant disease presenting with pancreatic neuroendocrine tumors (pNETs), parathyroid tumors, or pitu-itary 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 treat-ment is surgical resection. However, in the case of a func-tioning 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 recom-mended. 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 ef-fective in phase III clinical trials. (Gut Liver 2012;6:287-294)
Current Status of the Diagnosis and Management of Pancreatic Neuroendocrine Tumors in Japan
Tetsuhide Ito,Masami Miki,Keijiro Ueda,Lingaku Lee,Ken Kawabe,Hisato Igarashi,Nao Fujimori,Kazuhiko Nakamura,Kohei Yasunaga,Robert T. Jensen,Takao Ohtsuka,Yoshihiro Ogawa Korean Society of Gastrointestinal Cancer 2016 Journal of digestive cancer reports Vol.4 No.2
The epidemiology of pancreatic neuroendocrine neoplasms (PNENs) in Asia has been clarified through epidemiological studies, including one conducted in Japan, and subsequently another in South Korea. As endoscopic ultrasonography (EUS) has become more widely accessible, endoscopic ultrasound-fine needle aspiration (EUS-FNA) has been performed in pancreatic tumors for which the clinical course was only monitored previously. This has enabled accurate diagnosis of pancreatic tumors based on the 2010 WHO classification; as a result, the number of patients with an accurate diagnosis has increased. Although surgery has been the standard therapy for PNENs, new treatment options have become available in Japan for the treatment of advanced or inoperable PNENs; of particular note is the recent introduction of molecular target drugs (such as everolimus and sunitinib) and streptozocin. Treatment for progressive PNENs needs to be selected for each patient with consideration of the performance status, degree of tumor differentiation, tumor mass, and proliferation rate. Somatostatin receptor (SSTR)-2 is expressed in many patients with neuroendocrine tumor. Somatostatin receptor scintigraphy (SRS), which can visualize SSTR-2 expression, has been approved in Japan. The SRS will be a useful diagnostic tool for locating neuroendocrine neoplasms, detecting distant metastasis, and evaluating therapy outcomes. In this manuscript, we review the latest diagnostic methods and treatments for PNENs.