Originally described as mammary analog secretory carcinoma (SC), SC of the salivary gland is a rare malignancy with morphologic and molecular similarities to SC of the breast. We present 2 children with salivary gland SC with the classic ETV6-NTRK3 gene fusion, including 1 with lymph node metastases. Both patients underwent surgical resection and were in remission 24 months postsurgery. One patient was additionally found to have synchronous papillary thyroid carcinoma with a TFG-MET fusion. A review of published cases highlights the expanding molecular profile and confirms the favorable course of salivary gland SC after surgical resection.

唾液腺 SC 最初被描述为乳腺类似分泌癌 (SC)，是一种罕见的恶性肿瘤，其形态和分子与乳腺 SC 相似。我们介绍了 2 名具有经典ETV6-NTRK3基因融合的唾液腺 SC 儿童，其中 1 名患有淋巴结转移。两名患者均接受了手术切除，并在术后 24 个月内得到缓解。另外还发现一名患者同时患有TFG-MET融合的甲状腺乳头状癌。对已发表病例的回顾强调了分子谱的扩大，并证实了手术切除后唾液腺 SC 的有利过程。