Bao Yongyang, Li Jiang, Zhu Yanbo
Departments of Pathology, and.
Oral Pathology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Am J Dermatopathol. 2018 Jul;40(7):531-535. doi: 10.1097/DAD.0000000000001062.
Mammary analog secretory carcinoma (MASC) of salivary gland is a recently described neoplasm that morphologically and immunohistochemically resembles secretory carcinoma of the breast. Genetically, both of them harbor ETV-6-NTRK-3 fusion rearrangement. One case of primary MASCs arising from the eyelid is reported. The patient was a 52-year-old man. Microscopically, the tumor exhibited nodular aggregation of solid, tubular, and microcystic/macrocystic structures. Characteristic "colloid-like" eosinophilic secretory material was present within intraluminal spaces. Immunohistochemically, the tumor cells were positive for mammaglobin, S-100, STAT5a, vimentin, GCDFP-15, AE1/AE3, EMA, and CK7 and were negative for DOG-1, CK5/6, and SMA. A dual color break-apart fluorescence in situ hybridization probe identified rearrangement of the ETV6 gene locus on chromosome 12. The patient had no history of breast or salivary gland tumor. The tumor was completely excised, and the patient has no evidence of recurrent disease or metastasis after 1-year follow-up. A diagnosis of primary MASC was rendered. MASC has never been reported occurring in ocular region. This type of secretory carcinoma probably originates from sweat glands or accessory lacrimal glands, Wolfring and Krause. This unique case expands the clinicopathologic landscape of MASCs for better characterization of this rare entity.
涎腺乳腺样分泌性癌(MASC)是一种最近描述的肿瘤,在形态学和免疫组织化学上类似于乳腺分泌性癌。在基因方面,两者都存在ETV-6-NTRK-3融合重排。本文报道1例起源于眼睑的原发性MASC。患者为一名52岁男性。显微镜下,肿瘤表现为实性、管状和微囊/大囊结构的结节状聚集。管腔内可见特征性的“胶样”嗜酸性分泌物质。免疫组织化学检查显示,肿瘤细胞乳腺珠蛋白、S-100、信号转导和转录激活因子5a(STAT5a)、波形蛋白、GCDFP-15、AE1/AE3、上皮膜抗原(EMA)和细胞角蛋白7(CK7)呈阳性,而DOG-1、细胞角蛋白5/6(CK5/6)和平滑肌肌动蛋白(SMA)呈阴性。双色分离荧光原位杂交探针检测到12号染色体上ETV6基因位点重排。患者无乳腺或涎腺肿瘤病史。肿瘤被完整切除,1年随访后患者无疾病复发或转移迹象。最终诊断为原发性MASC。此前从未有MASC发生于眼部区域的报道。这种分泌性癌可能起源于汗腺或副泪腺(Wolfring腺和Krause腺)。这一独特病例拓展了MASC的临床病理谱,有助于更好地认识这种罕见疾病。