Gasque Rodrigo Antonio, Zaietta Noelia, Del Bueno María Luján, Saenz Julieta, Chahdi Beltrame Magalí, Quiñonez Emilio Gastón, Mattera Francisco Juan
Hospital de Alta Complejidad en Red "El Cruce".
Unidad de Cirugía hepatobiliar compleja, pancreática y trasplante hepático, Hospital de Alta Complejidad en Red "El Cruce", Florencio Varela, Buenos Aires, Argentina.
Rev Fac Cien Med Univ Nac Cordoba. 2023 Jun 30;80(2):141-144. doi: 10.31053/1853.0605.v80.n2.40368.
Primary neuroendocrine tumors (NETs) of the bile duct are extremely rare and represent only 0.2-2% of all gastrointestinal NETs. Within the biliary system, the main bile duct is the most affected site. A 28-year-old man with a 6-month history of intermittent jaundice, pruritus, and choluria. MRCP, PET-CT and endoscopic ultrasound were performed. A well-differentiated neuroendocrine neoplasia was diagnosed. Complete resection of the main bile duct was performed with lymphadenectomy of the hepatic pedicle with Roux-en-Y hepaticojejunostomy, without complications. The patient had an adequate evolution and nowadays he’s disease-free. Primary neuroendocrine tumors of the bile duct are extremely rare. They may present clinically and radiologically similar to perihilar cholangiocarcinoma, which makes preoperative diagnosis difficult. Radical resection is indicated. Usually, they are well differentiated tumors, being the Ki-67 labeling index a reliable prognostic marker.
胆管原发性神经内分泌肿瘤（NETs）极为罕见，仅占所有胃肠道NETs的0.2%-2%。在胆道系统中，主要胆管是最常受累的部位。一名28岁男性，有6个月间歇性黄疸、瘙痒和胆尿病史。进行了磁共振胰胆管造影（MRCP）、正电子发射断层显像-X线计算机体层成像（PET-CT）和内镜超声检查。诊断为高分化神经内分泌肿瘤。对主要胆管进行了完整切除，并对肝蒂进行了淋巴结清扫，同时行 Roux-en-Y 肝空肠吻合术，无并发症发生。患者恢复良好，目前无病生存。胆管原发性神经内分泌肿瘤极为罕见。它们在临床和影像学上可能与肝门周围胆管癌相似，这使得术前诊断困难。建议行根治性切除术。通常，它们是高分化肿瘤，Ki-67标记指数是一个可靠的预后标志物。
