Department of Experimental and Clinical Medicine, Polytechnic University of Marche, Ancona 60126, Italy.
Department of Gastroenterology and Transplant, United Hospital of Marche, Ancona 60126, Italy.
World J Gastroenterol. 2023 Oct 14;29(38):5361-5373. doi: 10.3748/wjg.v29.i38.5361.
Intraductal papillary neoplasms of the bile duct (IPNBs) represent a rare variant of biliary tumors characterized by a papillary growth within the bile duct lumen. Since their first description in 2001, several classifications have been proposed, mainly based on histopathological, radiological and clinical features, although no specific guidelines addressing their management have been developed. Bile duct neoplasms generally develop through a multistep process, involving different precursor pathways, ranging from the initial lesion, detectable only microscopically, biliary intraepithelial neoplasia, to the distinctive grades of IPNB until the final stage represented by invasive cholangiocarcinoma. Complex and advanced investigations, mainly relying on magnetic resonance imaging (MRI) and cholangioscopy, are required to reach a correct diagnosis and to define an adequate bile duct mapping, which supports proper treatment. The recently introduced subclassifications of types 1 and 2 highlight the histopathological and clinical aspects of IPNB, as well as their natural evolution with a particular focus on prognosis and survival. Aggressive surgical resection, including hepatectomy, pancreaticoduodenectomy or both, represents the treatment of choice, yielding optimal results in terms of survival, although several endoscopic approaches have been described. IPNBs are newly recognized preinvasive neoplasms of the bile duct with high malignant potential. The novel subclassification of types 1 and 2 defines the histological and clinical aspects, prognosis and survival. Diagnosis is mainly based on MRI and cholangioscopy. Surgical resection represents the mainstay of treatment, although endoscopic resection is currently applied to nonsurgically fit patients. New frontiers in genetic research have identified the processes underlying the carcinogenesis of IPNB, to identify targeted therapies.
胆管内乳头状肿瘤（IPNBs）是一种罕见的胆管肿瘤变异，其特征是胆管腔内的乳头状生长。自 2001 年首次描述以来，已经提出了几种分类方法，主要基于组织病理学、影像学和临床特征，尽管尚未制定针对其管理的具体指南。胆管肿瘤通常通过多步过程发展，涉及不同的前体途径，从仅在显微镜下可见的初始病变、胆管上皮内肿瘤，到具有特征性的 IPNB 分级，直到最后阶段的浸润性胆管癌。需要进行复杂和先进的检查，主要依赖磁共振成像（MRI）和胆管镜检查，以做出正确的诊断，并确定适当的胆管绘图，以支持适当的治疗。最近提出的 1 型和 2 型的亚分类突出了 IPNB 的组织病理学和临床方面，以及它们的自然演变，特别关注预后和生存。包括肝切除术、胰十二指肠切除术或两者在内的积极手术切除是首选治疗方法，在生存方面取得了最佳结果，尽管已经描述了几种内镜方法。IPNBs 是具有高恶性潜能的新认识的胆管前体肿瘤。1 型和 2 型的新分类定义了组织学和临床方面、预后和生存。诊断主要基于 MRI 和胆管镜检查。手术切除是主要的治疗方法，尽管内镜切除目前适用于不适合手术的患者。遗传研究的新前沿已经确定了 IPNB 发生的致癌过程，以确定靶向治疗。
