Sasaki M, Nakanuma Y, Shimizu K, Izumi R
Second Department of Pathology, School of Medicine, Kanazawa University, Japan.
Pathol Int. 1995 Oct;45(10):781-6. doi: 10.1111/j.1440-1827.1995.tb03397.x.
A case of mucinous cholangiocarcinoma is reported. The patient was a 49 year old woman with the complaint of lumbago. Imaging examination disclosed a tumor 3.5 cm in diameter in the right hepatic lobe, which showed low density on computerized tomography scans and low signal intensity on T1-weighted magnetic resonance imaging (MRI) and high intensity on T2-weighted MRI. The hepatic tumor expanded rapidly and multiple pulmonary metastases and peritoneal dissemination developed. The patient died due to respiratory failure 5 months after the initial symptom. An autopsy, a massive tumor (9.5 cm in diameter) containing abundant mucus with several surrounding daughter nodules was found in the right hepatic lobe. Metastases were widespread. Histologically, the tumor was composed mainly of numerous lobulated mucus lakes in which adenocarcinoma cells floated. Admixed signet ring cells were also noted. These gross and histologic features differ from those in other mucin-producing hepatic tumors such as biliary papillomatosis and mucinous cystoadenocarcinoma. Immunohistochemically, the adenocarcinoma cells were strongly positive for carcinoembryonic antigen, Lewis Y, Tn, and T antigens and moderately positive for carbohydrate antigen 19-9, Lewis X, sialyl-Lewis X and sialyl-Tn antigen. Mature MUC1 mucin and core protein of MUC1 mucin were also expressed to varying degrees. The rapidly expanding, widespread metastases and poor prognosis found in the present case may be the clinicopathological features of mucinous cholangiocarcinoma.
本文报告一例黏液性胆管癌病例。患者为一名49岁女性，主诉腰痛。影像学检查发现右肝叶有一个直径3.5厘米的肿瘤，在计算机断层扫描上呈低密度，在T1加权磁共振成像（MRI）上呈低信号强度，在T2加权MRI上呈高信号强度。肝脏肿瘤迅速增大，并出现多发肺转移和腹膜播散。患者在出现初始症状5个月后因呼吸衰竭死亡。尸检发现右肝叶有一个巨大肿瘤（直径9.5厘米），含有大量黏液，周围有几个子结节。转移广泛。组织学上，肿瘤主要由大量分叶状黏液湖组成，腺癌细胞漂浮其中。还可见混合的印戒细胞。这些大体和组织学特征与其他产生黏液的肝脏肿瘤如胆管乳头状瘤病和黏液性囊腺癌不同。免疫组化显示，腺癌细胞癌胚抗原、Lewis Y、Tn和T抗原呈强阳性，糖类抗原19-9、Lewis X、唾液酸化Lewis X和唾液酸化Tn抗原呈中度阳性。成熟的MUC1黏蛋白和MUC1黏蛋白的核心蛋白也有不同程度的表达。本病例中发现的肿瘤迅速增大、广泛转移及预后不良可能是黏液性胆管癌的临床病理特征。
