• Masaru Ogawa, Satoshi Yokoo, Takahiro Yamaguchi, Keisuke Suzuki, Mai Seki-Soda, Takahiro Shimizu, Jun Kurihara, and Takaya Makiguchi.
• Department of Oral and Maxillofacial Surgery, and Plastic Surgery, Gunma University Graduate School of Medicine, 4-39-15 Showa-machi, Maebashi, Gunma, Japan.
• Medicine (Baltimore). 2021 Dec 23; 100 (51): e28390e28390.

IntroductionSecretory carcinoma (SC) is a malignancy of the salivary glands, which is similar to SC of the breast regarding its association with neurotrophic tyrosine receptor kinase fusion-positive gene. SC is a recently described salivary gland tumor, and there are a few reports describing oral minor salivary gland-derived SC. We reported two cases of SC in the oral cavity and reviewed the literature.Patient ConcernsThe patients included a 65-year-old Japanese woman who presented with a mass of the upper lip and an 84-year-old Japanese man who presented with a mass on the buccal mucosa.DiagnosisDiagnosis was based on histomorphological and immunohistochemical findings and identification of a specific translocation of the ETS variant 6-neurotrophic receptor tyrosine kinase 3 gene fusion. Case 1 was finally diagnosed using reverse transcription-polymerase chain reaction with formalin-fixed paraffin-embedded tissue samples, while case 2 was diagnosed using fluorescence in situ hybridization analysis.Interventions And OutcomesIn case 1, excisional biopsy was done and there was no recurrence observed in five-year follow-up. In case 2, tumor resection was done and there was no recurrence observed in two-year follow-up.ConclusionIt is highly likely for many cases of SC to be initially diagnosed as acinic cell carcinoma (AciCC) owing to their similar histological findings. The treatment strategy for minor salivary gland-originated SC is similar to that of AciCC; however, SC is often highly malignant and involves a high risk of cervical lymph node metastasis. Thus, establishing an accurate diagnosis together with pathologists and confirming the presence of the ETS variant 6-neurotrophic receptor tyrosine kinase 3 fusion gene using genetic analysis is important.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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