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.