Foucar E, Kaplan L R, Gold J H, Kiang D T, Sibley R K, Bosl G
Gastroenterology. 1979 Aug;77(2):347-53.
A patient with an unresectable well-differentiated bile duct tumor who survived for 15 yr after biopsy diagnosis is presented. Histologic examination of the tumor revealed bland features of bile duct adenoma despite extensive spread within the liver. Over its subsequent course, the tumor progressively replaced the liver, achieving huge size, although there was no evidence of metastases until shortly before the patient's death. This clinical course was very unusual for either bile duct adenoma or cholangiocarcinoma, but would be more characteristic of another tumor of intrahepatic bile duct origin, the biliary cystadenoma. However, this latter diagnosis was excluded with both gross and microscopic pathologic criteria. Evidence is presented to support classification of this tumor as an unusual varient of peripheral cholangiocarcinoma which requires correlation of the clinical and pathologic findings for correst diagnosis.
本文报告了一例不可切除的高分化胆管肿瘤患者，活检诊断后存活了15年。肿瘤的组织学检查显示，尽管在肝脏内广泛扩散，但具有胆管腺瘤的温和特征。在其后续病程中，肿瘤逐渐取代肝脏，体积巨大，尽管直到患者死亡前不久才出现转移迹象。这种临床病程对于胆管腺瘤或胆管癌来说都非常不寻常，但更符合肝内胆管起源的另一种肿瘤——胆管囊腺瘤的特征。然而，根据大体和显微镜下病理标准排除了后者的诊断。有证据支持将该肿瘤分类为外周胆管癌的一种不寻常变体，正确诊断需要临床和病理结果的关联。
