报道了一位女性患者出现右侧颌下腺病变的细胞学诊断，诊断为黏液表皮样癌。患者接受了腮腺全切除术和颈部上韧带下淋巴清扫术。手术样本检测结果显示ETV6-NTRK3基因融合阳性，最终诊断为分泌性癌（SC）。世界卫生组织在2017年将分泌性癌从乳腺模拟分泌性癌（MASC）改名。到2019年为止，只报道了649例。虽然细胞学改变与腺泡细胞癌和黏液表皮样癌等其他肿瘤相似，ETV6-NTRK3基因重排是分泌性癌的病理特征。尽管通常发展缓慢，且处于低分期阶段，分泌性癌在局部复发和淋巴结受累方面的发生率较腺泡细胞癌更高。手术治疗是治疗的金标准。由于2017年世界卫生组织的新分类，分泌性癌的真实患病率可能被低估。尽管细胞学分析不能将分泌性癌与其他恶性肿瘤区分开来，但建议进行染色体检查。低级别的分泌性癌一旦诊断，完全的手术切除有利于良好的预后。版权所有 © 2023。由Elsevier Masson SAS出版。

A woman presented a right submandibular gland lesion with cytologic diagnosis of mucoepidermoid carcinoma. Patient underwent sialoadenectomy en bloc with supraomohyoid neck dissection. Positivity for ETV6-NTRK3 genes fusion on surgical sample led to final diagnosis of secretory carcinoma (SC). Secretory carcinoma has been renamed by WHO in 2017 from mammary-analogue-secretory carcinoma (MASC). Only 649 have been reported until 2019. While cytologic alteration are shared with other neoplasms as the acinic cell and mucoepidermoid carcinomas, ETV6-NTRK3 rearrangement is pathognomonic of SC. Although usually indolent and with low-stage presentation, SC has higher rate of local recurrences and nodal involvement than ACC. Surgical treatment represent the gold standard. Real prevalence of SC is probably underestimated due to the recent WHO 2017 reclassification. While cytologic analysis does not allow to discriminate SC from other malignancies, chromosomal examination is recommended. When low-grade SC is diagnosed, complete surgical resection assures good prognosis.Copyright © 2023. Published by Elsevier Masson SAS.