Fang Yichen, O'Leary Catherine, Lowe Hannah, Carpenter Danielle H, Randall Henry B, Nazzal Mustafa D
Department of Surgery, Saint Louis University School of Medicine, Saint Louis, USA.
Department of Medicine, Saint Louis University School of Medicine, Saint Louis, USA.
Cureus. 2024 Sep 25;16(9):e70177. doi: 10.7759/cureus.70177. eCollection 2024 Sep.
Biliary intraepithelial neoplasm (BilIN) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are recognized as the two main precursors to biliary carcinoma. Surgical resection is the mainstay of treatment, with pancreaticoduodenectomy for extrahepatic biliary duct disease involving the pancreatic or distal portion of the bile duct, and bile duct and liver resection for perihilar and intrahepatic bile duct involvement. For diffuse IPMN-B with involvement of the entirety of the biliary epithelium of both intrahepatic and extrahepatic biliary systems, there is no well-documented consensus on treatment due to its rarity. Therefore, we present a case of a 56-year-old male with diffuse IPMN-B managed with combined orthotopic liver transplant and pancreaticoduodenectomy. As such, the clinical presentation, diagnostics, and unique course of intervention for our patient are described. This case provides insight into the topic of managing IPMN-Bs, particularly with diffuse biliary tree involvement. With such a rare disease with wildly varied presentations, consensus on a set treatment algorithm is nearly impossible to establish. This case describes one treatment pathway we found to be successful.
胆管上皮内瘤变（BilIN）和胆管内乳头状黏液性肿瘤（IPMN-B）被认为是胆管癌的两种主要癌前病变。手术切除是主要的治疗方法，对于累及胰腺或胆管远端的肝外胆管疾病行胰十二指肠切除术，对于累及肝门和肝内胆管的病变行胆管和肝切除术。对于累及肝内和肝外胆管系统全部胆管上皮的弥漫性IPMN-B，由于其罕见性，目前尚无充分记录的治疗共识。因此，我们报告一例56岁男性弥漫性IPMN-B患者，采用原位肝移植联合胰十二指肠切除术进行治疗。本文描述了该患者的临床表现、诊断及独特的干预过程。该病例为IPMN-B的治疗，尤其是弥漫性胆管树受累的治疗提供了见解。对于这种临床表现差异很大的罕见疾病，几乎不可能建立一套固定的治疗算法共识。本病例描述了一条我们发现成功的治疗途径。
