Research advances in intraductal papillary mucinous neoplasm of the bile tract
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摘要: 胆管导管内乳头状黏液性肿瘤(IPMN-B)是发生在胆管系统的一类特殊肿瘤,其临床表现及影像学均无特异性,术前诊断困难,往往于术中发现胆管内充盈大量黏液时考虑本病而被迫临时改变手术方案。由于其发病率低,临床医师对其缺乏认识。综述了近年来IPMN-B的病因与分型、临床特征、鉴别诊断、治疗及预后等方面研究进展,以期提高临床医师对IPMN-B的诊疗水平。Abstract: Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is a special type of tumor occurring in the biliary system, with no specific clinical manifestations and imaging findings, and thus it is difficult to diagnose before surgery. It is often considered when a large amount of mucus is found in the bile duct during surgery and then it is forced to change the surgical plan. Clinicians lack an understanding of this disease due to its low incidence rate. This article reviews the recent research advances in the etiology and classification, clinical features, differential diagnosis, treatment, and prognosis of IPMN-B, so as to improve the clinical diagnosis and treatment of IPMN-B among clinicians.
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Key words:
- Bile Duct Neoplasms /
- Therapeutics /
- Prognosis
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表 1 IPMN-B的免疫组化分型
分型 MUC1 MUC2 MUC5AC MUC6 肠型 - + + - 胃型 - - + + 胰胆管型 + - + - 嗜酸性细胞型 + - + + 表 2 IPMN-B的影像学分型
分型 特征 典型IPMN-B 胆管内肿瘤灶和肿瘤上、下游胆管均明显扩张 囊性IPMN-B 胆管呈局限性囊状扩张,表现为囊性肿块,囊内可见瘤灶,可单发或多发 无肿块型IPMN-B 肝内外胆管均明显扩张,未见明显瘤灶和囊性肿块 侵袭型IPMN-B 瘤体不仅向腔内乳头状生长而且突破胆管壁向外浸润胆管周围组织 -
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