目的 研究涎腺分泌性癌(secretory carcinoma of salivary gland,SCSG)的临床表现、病理学特点、鉴别诊断及预后.方法 收集2021年1月-2023年9月新疆医科大学第一附属医院病理科确诊的8例SCSG,从组织学、免疫组织化学及分子检测多方面研究分析.结果 8例SCSG患者男女各4例,年龄21岁-84岁,中位年龄51岁,肿瘤直径1.5cm-6.5cm,临床上以缓慢生长的无痛性肿块为首发症状,所有患者均行手术切除.组织学以微囊状、管状-乳头状或实性结构为主.8例弥漫表达细胞角蛋白(CK)7和S-100蛋白,6例乳腺球蛋白阳性、1例部分阳性及1例仅个别细胞阳性;6例pan-Trk阳性,1例阴性,GCDFP15 3例阴性、1例部分阳性及1例个别细胞阳性;SOX-10阳性3例、2例阴性;Ki-67阳性指数3%-20%.8例均有ETV6-NTRK3融合基因信号.结论 SCSG是一种较罕见的低度恶性涎腺源性肿瘤,具有典型组织学和免疫表型,pan-Trk免疫组织化学可能与NTRK融合相关,ETV6-NTRK3基因重排不但具有诊断意义,同时Trk靶向治疗有望在临床治疗中发挥更大作用.
Secretory carcinoma of salivary gland:a clinicopathological and prognostic analysis of eight cases
Objective To study the clinical manifestations,pathological features,molecular features,differential diagnosis and prognosis of secretory carcinoma of salivary gland(SCSG).Methods Eight cases of SCSG diagnosed in the Department of Pathology,the First Affiliated Hospital of Xinjiang Medical University from January 2021 to September 2023 were collected and analyzed in terms of histological morphology,immunohistochemistry and molecular detection.Results Among the 8 patients,there were 4 males and 4 females,aged 21-84 years old,with a median age of 51 years.The maximum diameter of the tumors ranged from 1.5 to 6.5 cm.Clini-cally,slow-growing painless mass was the first symptom,and all patients underwent surgical resection.Histopathological,the tumor was characterized by microcystic,tubular-Papillary or solid structures.Immunohistochemistry showed diffuse expression of CK7 and S-100 in all cases,mammaglobin(6/8)positive,(1/8)partially positive and(1/8)some individual cells positive,pan-Trk(6/8)posi-tive and(1/8)negative,GCDFP15(3/8)negative and(1/8)partially positive and(1/8)some individual cells positive,SOX-10(3/8)positive and(2/8)negative,and Ki-67 index was 3%-20%.All 8 cases had ETV6-NTRK3 gene fusion.Conclusions SCSG is a relatively rare low-grade malignant salivary gland tumor,with typical histological morphology and immunophenotype,Pan-Trk Immuno-histochemistry may be related to NTRK fusion,ETV6-NTRK3 gene rearrangement is not only of diagnostic significance,but also Trk-targeted therapy is expected to play greater role in clinical treatment.
Secretory carcinoma of salivary glandImmunohistochemistryGene fusionDiagnosis