软腭唾液腺分泌性癌1例并文献复习

Secretory carcinoma of salivary glands of the soft palate:A case report and literature review

唾液腺分泌性癌(secretory carcinoma of salivary glands,SCSG)是一种罕见的恶性唾液腺肿瘤。2023年2月,大理大学第一附属医院收治1例左侧软腭见1个肿物的35岁女性患者。显微镜下该肿物形成微囊状、小管状结构,腔隙内充满嗜酸性胶样分泌物。免疫组织化学(immunohistochemistry,IHC)示:广谱原肌球蛋白受体激酶(pantropomyosin receptor kinase,pan-TRK)、乳腺球蛋白(mammaglobin)、S100钙结合蛋白(S100 calcium binding protein,S100)均阳性,钙激活氯通道蛋白1(anoctamin-1,ANO1)阴性。荧光原位杂交(fluorescence in situ hybridization,FISH)示:ETS变异转录因子6(ETS variant transcription factor 6,ETV6)基因断裂阳性,ETV6-神经酪氨酸激酶受体3(neurotrophic receptor tyrosine kinase 3,NTRK3)基因融合阳性,MET原癌基因受体酪氨酸激酶(MET proto-oncogene,receptor tyrosine kinase,MET)基因断裂阴性。患者最终被诊断为SCSG,随后接受了“左侧腭部肿物切除术+邻近皮瓣修复术”,截至2024年2月未见复发或转移。本文回顾性分析1例发生于软腭的SCSG,并总结此前国内外发生于软腭的SCSG共12例。

Secretory carcinoma of salivary glands(SCSG)is a rare malignant salivary gland tumor.In February 2023,a 35-year-old female patient was admitted to the First Affiliated Hospital of Dali University with a mass in the left soft palate.Under the microscope,the mass formed microcystic and tubular structures,which filled with eosinophilic gelatinous secretions.Immunohistochemistry(IHC):Pan-tropomyosin receptor kinase(pan-TRK),mammaglobin and S100 calcium binding protein(S100)were positively expressed in tumor cells,while anoctamin-1(ANO1)was negative.Fluorescence in situ hybridization(FISH):ETS variant transcription factor 6(ETV6)gene disruption and ETV6-neurotrophic receptor tyrosine kinase 3(NTRK3)gene fusion were positive,but MET proto-oncogene,receptor tyrosine kinase(MET)gene disruption was negative.The patient was eventually diagnosed with SCSG,then accepted‘Left Palatal Mass Resection and Adjacent Flap Repair’and did not appear recurrence or metastasis in February 2024.In this study,a case of SCSG in soft palate was retrospectively analyzed,and a total of 12 cases of SCSG in soft palate were summarized.