Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
Department of Pathomorphology, Medical University of Warsaw, Warsaw, Poland.
Adv Med Sci. 2021 Mar;66(1):138-147. doi: 10.1016/j.advms.2021.01.005. Epub 2021 Feb 6.
Intraductal papillary neoplasm of the bile ducts is a rare tumor type. Management decisions are currently based upon a small case series. The authors have large own experience with IPNB.
The review aims at reporting on clinicopathological features of IPNB in order to provide guidance for management.
We searched PubMed, Medline, Microsoft Academic and Embase databases to identify studies of relevance. The analysis of own experience was also included.
We analyzed 59 retrospective series and 25 cases from authors' clinical experience. The main sign was jaundice and cholangitis, 33% and 48%, respectively. CT's were performed in 63-76% and MR in 40-56%. Intraductal mass was found in 31-32% and duct dilatation in 27-30%. Endoscopic Retrograde Cholangio-Pancreatography (ERCP) was performed in 48-62%. IPNB with invasive carcinoma was found in 35.7-60% and IPNB with intraepithelial neoplasia in 36-60%. Histopathological confirmation before surgery was rare. The main treatment of IPNB is resection, in our material, both, hepatectomy and hepatectomy plus bile duct resections were performed in 40% of patients. The percentage of postoperative complications was 20%. The 5-year survival rate of all IPNB's patients was 53.6%; in patients with associated invasive carcinoma - 22.2% and without invasive carcinoma - 100% (p = 0.001).
Early surgery is advisable for radiologically suspected IPNB. The results of treatment depend on histopathology. They are worse at intraductal invasive carcinoma than at neoplasm with neoplasia.
胆管内乳头状肿瘤是一种罕见的肿瘤类型。目前的治疗决策基于小样本病例系列。作者有丰富的胆管内乳头状肿瘤经验。
本综述旨在报告胆管内乳头状肿瘤的临床病理特征,为治疗提供指导。
我们检索了 PubMed、Medline、Microsoft Academic 和 Embase 数据库,以确定相关研究。同时还分析了作者的临床经验。
我们分析了 59 个回顾性系列和 25 例作者的临床经验。主要症状是黄疸和胆管炎,分别占 33%和 48%。63%-76%的患者行 CT 检查,40%-56%的患者行 MR 检查。31%-32%的患者发现胆管内肿块,27%-30%的患者胆管扩张。48%-62%的患者行内镜逆行胰胆管造影术(ERCP)。35.7%-60%的患者发现有浸润性癌的胆管内乳头状肿瘤,36%-60%的患者发现有上皮内肿瘤的胆管内乳头状肿瘤。术前组织学证实罕见。胆管内乳头状肿瘤的主要治疗方法是切除,在我们的资料中,40%的患者行肝切除术和肝切除术加胆管切除术。术后并发症的发生率为 20%。所有胆管内乳头状肿瘤患者的 5 年生存率为 53.6%;伴浸润性癌的患者为 22.2%,无浸润性癌的患者为 100%(p=0.001)。
对于影像学怀疑的胆管内乳头状肿瘤,早期手术是明智的。治疗结果取决于组织病理学。有浸润性癌的胆管内乳头状肿瘤比有上皮内肿瘤的胆管内乳头状肿瘤的结果更差。