Department of Anatomic Pathology, Hirosaki University School of Medicine and Hospital, Aomori, Japan.
Pathol Int. 2020 Sep;70(9):591-601. doi: 10.1111/pin.12954. Epub 2020 May 31.
Clear cell carcinoma (CCC) is a distinct histologic type of ovarian carcinoma. CCC is more frequent in Japan than in the Western world. CCC is chemo-resistant and often associated with paraneoplastic thromboembolism. Histologically, CCC is characterized by both cancer cells and stromas, being concordant with the cytological features. Clear cells contain abundant glycogen. Hepatocyte nuclear factor-1β is a specific marker of CCC, and is likely to be involved in glucose metabolism. Extracellular matrix (ECM)-deposited stroma and plasma cell-rich inflammatory stroma are characteristic stromas of CCC. Studies using CCC cell lines showed that CCC cells produce ECMs and stimulate plasma cell differentiation in a paracrine manner. Most CCCs, as well as endometrioid carcinomas, originate from ovarian endometriosis. This is supported by molecular genetic data, although it remains unclear why different histologic types originate from the same precursor. CCC and endometrioid carcinoma are Lynch syndrome-associated ovarian carcinomas. Recent comprehensive studies indicate that CCC is distinct not only in terms of histology but also in genomics, epigenomics and transcriptomics. This review summarizes the pathology of ovarian CCC along with a basic view based on cultured cells, and refers to recent genetic and omic data.
透明细胞癌（CCC）是卵巢癌的一种独特组织学类型。CCC 在日本比在西方世界更为常见。CCC 对化疗有抗药性，并且常与副肿瘤性血栓栓塞相关。组织学上，CCC 的特征是既有癌细胞又有基质，与细胞学特征一致。透明细胞富含糖原。肝细胞核因子-1β（Hepatocyte nuclear factor-1β）是 CCC 的特异性标志物，可能参与葡萄糖代谢。细胞外基质（extracellular matrix，ECM）沉积基质和富含浆细胞的炎症基质是 CCC 的特征性基质。使用 CCC 细胞系的研究表明，CCC 细胞以旁分泌的方式产生 ECM 并刺激浆细胞分化。大多数 CCC 以及子宫内膜样癌都起源于卵巢子宫内膜异位症。尽管分子遗传学数据支持这一点，但尚不清楚为什么不同的组织学类型起源于相同的前体。CCC 和子宫内膜样癌是林奇综合征相关的卵巢癌。最近的全面研究表明，CCC 不仅在组织学上，而且在基因组学、表观基因组学和转录组学上都具有独特性。这篇综述总结了卵巢 CCC 的病理学，以及基于培养细胞的基本观点，并提到了最近的遗传学和组学数据。
