Pais-Costa Sergio Renato, Martins Sandro J, Araújo Sergio L M, Lima Olímpia A T, Paes Marcio A, Guimarães Marcio L
Hospital Santa Lucia and.
Rare Tumors. 2011 Oct 21;3(4):e36. doi: 10.4081/rt.2011.e36. Epub 2011 Nov 7.
Extrahepatic bile duct cancer is an uncommon disease, and few cases are curable by surgery. We report a case of extrahepatic biliary cystadenocarcinoma (BCAC) associated with atrophy of the left hepatic lobe. A 54-year old male was admitted with painless obstructive jaundice and a hepatic palpable mass noticed one month before presentation. Liver functions tests were consistent with cholestatic damage and serum carbohydrate antigen 19.9 (CA 19-9) was increased before treatment. Magnetic resonance imaging (MRI) disclosed dilatation of the left hepatic bile duct with irregular wall thickening close to the hepatic confluence, and atrophy of left hepatic lobe. The patient was submitted to en bloc extended left hepatectomy with resection of caudate lobe, hilar lymphadenectomy, and suprapancreatic biliary tree resection. All surgical margins were grossly negative, and postoperative course was uneventful, except for a minor bile leak. The patient was discharged on the 15th postoperative day; he is alive without tumor recurrence one year after primary therapy. Although technically challenging, extended en bloc resection is feasible in adults with extrahepatic BCAC and can improve survival with acceptable and manageable morbidity.
肝外胆管癌是一种罕见疾病，很少有病例能通过手术治愈。我们报告一例与左肝叶萎缩相关的肝外胆管囊腺癌（BCAC）。一名54岁男性因无痛性梗阻性黄疸入院，就诊前一个月发现肝脏可触及肿块。肝功能检查结果与胆汁淤积性损伤相符，治疗前血清糖类抗原19.9（CA 19-9）升高。磁共振成像（MRI）显示左肝内胆管扩张，肝门汇合处附近管壁不规则增厚，左肝叶萎缩。该患者接受了包括尾状叶切除、肝门淋巴结清扫和胰上胆管树切除的整块扩大左肝切除术。所有手术切缘肉眼观均为阴性，术后病程顺利，仅出现轻微胆漏。患者术后第15天出院；初次治疗一年后存活，无肿瘤复发。尽管技术上具有挑战性，但整块扩大切除术对于患有肝外BCAC的成年人是可行的，并且可以在可接受和可控制的发病率情况下提高生存率。
