University of Texas, Southwestern Dallas, USA.
J Gastrointestin Liver Dis. 2009 Mar;18(1):95-7.
A 3-year old male presented with complaints of pruritus, abdominal pain for 3 weeks and jaundice. Stools were acholic. There was jaundice, liver palpable 3 cm below right costal margin, no ascites or palpable masses. Serology revealed albumin 2.9 g/dl; ammonia of 31 mmol/l; elevated conjugated bilirubin, GGT, ALT, AST and alkaline phosphatase; alpha fetoprotein 1.3 ngm/ml; BhCG 9.1 IU/; PT 12.3 secs, INR 0.9; negative hepatitis A,B,C serology. CT scan showed a non-calcified heterogeneously enhancing mass centered at the liver hilum. MRCP showed a large heterogeneously enhancing, partially solid mass in the region of the porta hepatic. Liver biopsy revealed patternless proliferation of polymorphic oval to spindled shaped neoplastic cells. There was bile ducts distortion. Immunohistochemistry revealed positivity for vimentin, desmin.These findings were diagnostic for biliary rhabdomyosarcoma.There was no evidence of metastasis. Chemotherapy was initiated. Repeat imaging 6 months after initiation of treatment showed improvement in the degree of intrahepatic ductal dilatation and decrease in tumor bulk size. Rhabdomyosarcoma is the most common malignant tumor of the biliary tree in childhood. It is difficult to diagnose and delayed diagnosis influences the prognosis.
一位 3 岁男性因瘙痒、腹痛 3 周和黄疸就诊。大便呈陶土色。存在黄疸，肝脏在右肋缘下可触及 3cm，无腹水或可触及的肿块。血清学检查显示白蛋白 2.9g/dl；氨 31mmol/L；结合胆红素、GGT、ALT、AST 和碱性磷酸酶升高；甲胎蛋白 1.3ng/ml；BhCG9.1IU/；PT12.3 秒，INR0.9；肝炎 A、B、C 血清学阴性。CT 扫描显示肝门处不均匀强化的非钙化性肿块。MRCP 显示肝门区大的不均匀强化、部分实性肿块。肝活检显示多形性椭圆形至梭形成瘤细胞的无模式增殖。胆管变形。免疫组织化学显示波形蛋白和结蛋白阳性。这些发现诊断为胆管横纹肌肉瘤。无转移证据。开始化疗。治疗开始 6 个月后重复成像显示肝内胆管扩张程度改善，肿瘤体积缩小。横纹肌肉瘤是儿童胆道最常见的恶性肿瘤。它难以诊断，延迟诊断会影响预后。
