臨牀消化器内科 Vol.29 No.13(1)


特集名 膵管内乳頭粘液性腫瘍(IPMN)の診療の現況
題名 IPMNの病理
発刊年月 2014年 12月
著者 古川 徹 東京女子医科大学統合医科学研究所
【 要旨 】 膵管内乳頭粘液性腫瘍(IPMN)は膵管拡張を主徴とし,膵管内に粘液産生を示す乳頭状の腫瘍性上皮の増生を見る疾患である.上皮異型の程度,浸潤癌の有無により膵管内乳頭粘液性腺腫,非浸潤性膵管内乳頭粘液性腺癌,浸潤性膵管内乳頭粘液性腺癌に分けられ,また,腫瘍性上皮の特徴的な乳頭状構造より胃型,腸型,膵胆道型,好酸性細胞型に分けられる.これら異型度,亜型は臨床徴候とよく関連し,多様なIPMNを的確に診断することに有用である.IPMNに特異的な分子異常としてGNASRNF43変異が同定された.IPMN発生進展の分子機序を明らかにすることによりIPMNに特異的な分子診療が開発できる可能性がある.
Theme The Current State of Management of Intraductal Papillary Mucinous Neoplasm (IPMN) of the Pancreas
Title Pathology of IPMN
Author Toru Furukawa Institute for Integrated Medical Sciences, Tokyo Womenʼs Medical University
[ Summary ] Intraductal papillary mucinous neoplasm of the pancreas (IPMN) manifests a dilated duct filled with mucin. Neoplastic cells line the dilated duct and show complex papillary architecture. According to dysplastic grade and invasive nature, the neoplasms are classified into IPMN with lowgrade dysplasia, IPMN with high-grade dysplasia, and IPMN with an associated invasive carcinoma. The neoplasms are also classified by the morphological architecture of neoplastic papillae into gastric type, intestinal type, pancreatobiliary type, and oncocytic type. The dysplastic grade and morphological type are well associated with clinicopathological features of IPMN including patients' prognosis. Mutations in GNAS and RNF43 have been identified as specific molecular alterations in IPMN. The notion of molecular alterations may pave the way to develop new molecular medications in IPMN.
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