title: Advances in the diagnosis and treatment of pancreatic neuroendocrine neoplasms in Japan.
abstract: Several new developments have occurred in the field of pancreatic neuroendocrine neoplasm (PNEN) recently in Japan. First, the utility of chromogranin A (CgA), useful for the diagnosis and monitoring of the treatment response of neuroendocrine neoplasm (NEN), has been demonstrated in Japan. For PNEN diagnosis and treatment, grading and correct histological diagnosis according to the WHO 2010 classification is important. Regarding the histological diagnosis, the advent of endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) has enabled correct pathological diagnosis and suitable treatment for the affected tissue. Furthermore, EUS-FNA has also facilitates the assessment of the presence or absence of gene mutations. In addition, patients who have a well-differentiated neuroendocrine tumor (NET) showing a Ki-67 index of higher than 20 % according to the WHO 2010 classification, have also been identified, and their responses to treatment were found to be different from those of patients with poorly differentiated neuroendocrine carcinoma (NEC). Therefore, the concept of NET G3 was proposed. Additionally, somatostatin receptor type 2 is expressed in several cases of NET, and somatostatin receptor scintigraphy (111In-octreoscan) has also been approved in Japan. This advancement will undoubtedly contribute to the localization diagnosis, the identification of remote metastasis, and assessments of the treatment responses of PNEN. Finally, regarding the treatment strategy for PNEN, the management of liver metastasis is important. The advent of novel molecular-targeted agents has dramatically improved the prognosis of advanced PNEN. Multimodality therapy that accounts for the tumor stage, degree of tumor differentiation, tumor volume, and speed of tumor growth is required.
authors.affiliation: Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. itopapa@intmed3.med.kyushu-u.ac.jp.
authors.forename: Tetsuhide
authors.initials: T
authors.lastname: Ito
authors.affiliation: Department of Gastroenterology, Aichi Cancer Center Hospital, Nagoya, Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Hijioka
authors.affiliation: Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Masui
authors.affiliation: Department of Pathology, Tohoku University Hospital, Sendai, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Kasajima
authors.affiliation: Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Nakamoto
authors.affiliation: Department of Oncology, Graduate School of Medicine, Yokohama City University, Yokohama, Japan.
authors.forename: Noritoshi
authors.initials: N
authors.lastname: Kobayashi
authors.affiliation: Department of Surgery, Kansai Electric Power Hospital, Osaka, Japan.
authors.forename: Izumi
authors.initials: I
authors.lastname: Komoto
authors.affiliation: Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hijioka
authors.affiliation: Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
authors.forename: Lingaku
authors.initials: L
authors.lastname: Lee
authors.affiliation: Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
authors.forename: Hisato
authors.initials: H
authors.lastname: Igarashi
authors.affiliation: Digestive Diseases Branch, National Institutes of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Robert Thomas
authors.initials: RT
authors.lastname: Jensen
authors.affiliation: Neuroendocrine Tumor Center, Kansai Electric Power Hospital, Osaka, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Imamura
mesh_terms: D053379:Chromogranin A / Q000032:analysis; D003131:Combined Modality Therapy; D061765:Endoscopic Ultrasound-Guided Fine Needle Aspiration / Q000379:methods; D006801:Humans; D007564:Japan; D060787:Neoplasm Grading; D009367:Neoplasm Staging; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D011379:Prognosis
chemical_list: D053379:Chromogranin A
keywords: 111In-octreoscan; Chromogranin A; EUS-FNA; Molecular target therapy; NET G3; Neuroendocrine tumor; Pancreatic neuroendocrine neoplasm; Somatostatin receptor scintigraphy; WHO classification