肝脏的其他原发性上皮性肿瘤。

Other Primary Epithelial Neoplasms of the Liver.

作者信息

Szeto Wai, Mannan Rifat

机构信息

Department of Pathology, City of Hope National Medical Center, Duarte, CA.

出版信息

Adv Anat Pathol. 2025 Sep 1;32(5):338-348. doi: 10.1097/PAP.0000000000000494. Epub 2025 Apr 9.

Abstract

Primary liver carcinoma (PLC) is the sixth most common malignancy worldwide and the third leading cause of cancer-related mortalities. Hepatocellular carcinoma (HCC) is the most prevalent form of PLC, followed by intrahepatic cholangiocarcinoma (iCCA). In addition, there is a group of rarer PLCs that do not fit neatly into the HCC or iCCA categories. This review explores this heterogeneous group, including combined hepatocellular-cholangiocarcinoma (cHCC-CCA), intermediate cell carcinoma (ICC), mixed hepatocellular-neuroendocrine carcinoma, and undifferentiated primary liver carcinoma. cHCC-CCA is a rare subtype of PLC, characterized by both hepatocytic and cholangiocytic differentiation within the same tumor. The latest WHO classification (2019, fifth edition) redefined cHCC-CCA by eliminating the "stem cell subtypes" and emphasized that diagnosis should primarily rely on morphologic features, supported by immunohistochemical staining to better define subtypes. Intermediate cell carcinoma is a subtype of cHCC-CCA and is comprised of monomorphic tumor cells that exhibit characteristics intermediate between hepatocytes and cholangiocytes, with immunohistochemical expression of hepatocytic and cholangiocytic markers within the same cell. Another rare entity, combined HCC and neuroendocrine carcinoma (NEC), contains an admixture of HCC and NEC components within the same tumor. Undifferentiated primary liver carcinoma, on the other hand, lacks definitive lineage differentiation beyond an epithelial phenotype. These heterogeneous PLCs pose diagnostic challenges owing to their mixed/unusual histologic features and overlapping immunohistochemical markers. They tend to have poor prognoses, highlighting the critical importance of accurate and timely diagnosis.

摘要

原发性肝癌(PLC)是全球第六大常见恶性肿瘤,也是癌症相关死亡的第三大主要原因。肝细胞癌(HCC)是PLC最常见的形式,其次是肝内胆管癌(iCCA)。此外,还有一组较为罕见的PLC,它们并不完全符合HCC或iCCA的类别。本综述探讨了这一异质性群体,包括肝细胞-胆管癌(cHCC-CCA)、中间细胞癌(ICC)、混合性肝细胞-神经内分泌癌和未分化原发性肝癌。cHCC-CCA是PLC的一种罕见亚型,其特征是在同一肿瘤内同时具有肝细胞和胆管细胞分化。世界卫生组织最新分类(2019年,第五版)通过剔除“干细胞亚型”重新定义了cHCC-CCA,并强调诊断应主要依靠形态学特征,辅以免疫组织化学染色以更好地定义亚型。中间细胞癌是cHCC-CCA的一种亚型,由单形性肿瘤细胞组成,这些细胞表现出介于肝细胞和胆管细胞之间的特征,同一细胞内有肝细胞和胆管细胞标志物的免疫组织化学表达。另一种罕见实体,即合并HCC和神经内分泌癌(NEC),在同一肿瘤内含有HCC和NEC成分的混合。另一方面,未分化原发性肝癌除上皮表型外缺乏明确的谱系分化。这些异质性PLC因其混合/不寻常的组织学特征和重叠的免疫组织化学标志物而带来诊断挑战。它们往往预后较差,凸显了准确及时诊断的至关重要性。

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