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　　摘要：肠型胆管内乳头状肿瘤（intraductal papillary neoplasm of the bile duct，ilPNB）表现为柱状细胞，假复层排列、胞核雪茄状，胞质嗜碱性或双嗜染性，免疫组化染色CK20和/或CDX2弥漫表达。本研究旨在探讨该肿瘤的病理和遗传学特征。对34例ilPNB按解剖部位（胆管）进行了病理学检查，并与22例肠型胰腺导管内乳头状粘液性肿瘤（intraductal papillary mucinous neoplasm，ilPMN）进行对比分析。使用TruSight Tumor 26和二代测序法检测了21例经福尔马林固定、石蜡包埋的ilPNB患者组织标本中的26个体细胞突变基因。所有这些ilPNB病例被分成肝内（n=6）和肝外（n=28）两种类型。肝内型lPNB主要表现为单纯的绒毛-乳头状模式，而肝外型lPNB的乳头包括管状和/或绒毛状结构，且结构复杂，伴有明显重度异型增生。MUC5AC在肝内ilPNB和ilPMN中多广泛表达，而在肝外ilPNB中不表达。CD10在肝外IPNB中表达较多，而在肝内ilPNB和ilPMN中不表达。TP53和PIK3CA基因突变在肝外ilPNB 中常见，而在ilPMN 中罕见或缺失；KRAS和GNAS突变在肝内ilPNB和ilPMN中都较常见。肝内ilPNB呈绒毛-乳头状生长，其特征类似于ilPMN；而肝外ilPNB与ilPMN不同，乳头含有管状和/或绒毛状结构，组织学复杂，这些都提示，由于位于胆管树不同的位置，ilPNB 在肿瘤发生学方面存在差别。

　　关键词：胆管树，胆管内乳头状肿瘤，肠型，胰腺导管内乳头状粘液性肿瘤，二代测序

(Am J Surg Pathol 2019;43:1212-1220)

美国外科病理学杂志中文版2020年第一期摘要NO.3

(刘霞　翻译　贾永峰　审校）

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