Chulabhorn International College of Medicine, Thammasat University, Pathumthani, Thailand.
Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Pathol Int. 2020 Sep;70(9):661-670. doi: 10.1111/pin.12983. Epub 2020 Jul 7.
Cholangiocarcinoma is a malignant neoplasm originating from the biliary epithelium. Its incidence is highest in Southeast Asia, especially in Thailand. Mucinous intrahepatic cholangiocarcinoma (mucinous iCCA), characterized by an abundant extracellular mucin pool accounting for at least 50% of total tumor volume, is an extremely rare variant of such malignancy and is notorious for rapid progression and dismal prognosis. We conducted an 11-year retrospective analysis of resected mucinous iCCAs from our institution with a systematic review on mucinous iCCAs and combined hepatocellular-mucinous cholangiocarcinoma (cHCC-mCCA). There were four resected mucinous iCCA specimens at our institution (prevalence = 0.5%). Most of the patients were male. The clinicopathological characteristics were variable. The diagnosis of mucinous iCCAs could not be rendered without pathological evaluation. Either intraductal papillary neoplasm or biliary intraepithelial neoplasia was present in three out of four cases. One patient passed away at 11 months following liver resection. A total of 19 mucinous iCCAs and four cHCC-mCCAs from previously published literature were analyzed. The 1-year mortality rate of mucinous iCCAs from our series and published literature is 35%. The present study confirmed that mucinous iCCA is an exceedingly uncommon variant of iCCA. The differential diagnoses include metastatic carcinoma with mucinous component and cHCC-mCCA.
胆管癌是一种源自胆管上皮的恶性肿瘤。其发病率在东南亚最高，尤其是在泰国。黏液性肝内胆管癌（mucinous iCCA）是一种极其罕见的恶性肿瘤，其特征是丰富的细胞外黏液池，占肿瘤总体积的至少 50%。它以快速进展和预后不良而臭名昭著。我们对本机构切除的黏液性 iCCA 进行了 11 年的回顾性分析，并对黏液性 iCCA 和肝细胞癌-黏液性胆管细胞癌（cHCC-mCCA）进行了系统回顾。本机构有四个切除的黏液性 iCCA 标本（患病率=0.5%）。大多数患者为男性。临床病理特征各不相同。如果没有病理评估，就无法做出黏液性 iCCA 的诊断。四个病例中有三个存在胆管内乳头状肿瘤或胆管上皮内肿瘤。一名患者在肝切除后 11 个月去世。分析了之前发表的文献中的 19 个黏液性 iCCA 和 4 个 cHCC-mCCA。本系列和已发表文献中黏液性 iCCA 的 1 年死亡率为 35%。本研究证实，黏液性 iCCA 是 iCCA 非常罕见的一种变体。鉴别诊断包括伴黏液成分的转移性癌和 cHCC-mCCA。
