【摘要】 目的 探讨涎腺透明细胞癌(CCC)的临床病理学特征、诊断与鉴别诊断要点及预后。方法 收集河南省人民医院病理科2012-03—2021-11月诊断的4例涎腺CCC,观察其组织学形态、免疫表型及分子遗传学特征，随访患者并复习相关文献。结果 4例患者诊断时年龄46～71岁，平均58岁，男女比例为1∶1。3例发生于上腭，1例发生于颊部。肿瘤大小1～6 cm,平均3.3 cm。4例患者均行肿瘤扩大切除术，术后随访3～98个月均存活，1例复发并伴区域淋巴结转移。肿瘤细胞胞质透明或嗜酸性，呈巢状、条索状、小梁状及片状排列，伴致密的玻璃样变间质。特殊染色及免疫表型：肿瘤细胞PAS染色阳性而D-PAS染色阴性；CK7、CK5/6、p63和p40均呈阳性表达，S-100、SMA、Calponin、SOX-10、DOG1、PAX8和CA-Ⅸ均呈阴性，Ki-67增殖指数为2%～10%。4例FISH检测均存在EWSR1基因易位。结论 CCC是一种罕见的低级别涎腺癌，多发生于小涎腺，具有特征性的形态学表现及EWSR1基因易位，总体预后较好。更多还原

【Abstract】 Objective To investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of clear cell carcinoma(CCC) of the salivary gland. Methods Four cases of slivary CCC diagnosed in the Department of Pathology of Henan Provincial People′s Hospital from March 2012 to November 2021 were collected. The histological, immunohistochemical and molecular characteristics of CCC were observed. The patients were followed up and related literatures were reviewed. Results The age of the 4 patients at diagnosis was 46-71 years(mean 58 years), and the male to female ratio was 1∶1. Three cases occurred in the upper palate and one in the buccal region. The tumor size ranged from 1 to 6 cm, with an average of 3.3 cm. All 4 patients underwent expanded tumor resection and survived 3-98 months of follow-up. One patient relapsed with regional lymph node metastasis. The tumor cells with variably clear cytoplasm arranged in nests, cords, trabeculae and sheets in a dense hyalinized stroma. Special staining and immunophenotyping showed that tumor cells were PAS positive, CK7, CK5/6, p63 and p40 were positive, S-100, SMA, Calponin, SOX-10, DOG1, PAX8 and CA-Ⅸ were negative; Ki-67 proliferation index was 2%-10%. EWSR1 gene rearrangement was detected in all 4 patients. Conclusion CCC is a rare low-grade salivary gland carcinoma, mainly occurring in the minor salivary gland. It has characteristic histological features and EWSR1 gene fusion, and the overall prognosis is favorable.更多还原