生殖器汗管瘤39例临床及组织病理分析  

Clinical and histopathological analysis of genital syringoma: a report of 39 cases

作　　者：阚婷会 冯林[2] 卢阳[2] 张颖[2] 刁庆春 KAN Ting-hui;FENG Lin;LU Yang;ZHANG Ying;DIAO Qing-chun(Department of Dermatology,the Affiliated Hospital of Southwest Medical University,Luzhou 646000,China)

机构地区：[1]西南医科大学附属医院皮肤科,四川泸州646000 [2]重庆市中医院皮肤科,重庆400011

出　　处：《临床皮肤科杂志》2021年第12期709-712,共4页Journal of Clinical Dermatology

摘　　要：目的:总结39例生殖器汗管瘤的临床及组织病理学特点,进而提高对本病及其亚型的认识。方法:对重庆市中医院皮肤科门诊2010年1月—2019年9月间39例皮损位于外生殖器部位,组织病理诊断为汗管瘤患者的临床及组织病理资料进行回顾性分析。结果:39例患者中女38例(97.4%),男1例(2.6%),平均发病年龄33.8岁。临床诊断正确率仅61.5%,最易误诊为大汗腺痒疹。组织病理表现为真皮中上部实性上皮细胞团块及条索,可见管腔样结构,管壁为双层上皮细胞,多数管腔内见嗜伊红物质;15例(38.5%)患者可见粟丘疹样结构,囊内为疏松角质物,肿瘤团块周边不同程度纤维组织增生。免疫组化显示全部患者细胞团块及导管管腔细胞角蛋白(CK)5/6阳性,S-100蛋白阴性,导管管腔癌胚抗原(CEA)阳性;5例(12.8%)CK7散在阳性;并发粟丘疹样结构的患者(15例)中,15例(100%)囊壁CK5/6阳性,14例(93.3%)囊壁CEA阳性,CK7及S-100蛋白均为阴性表达。结论:生殖器汗管瘤好发于女性,临床上易误诊为大汗腺痒疹,组织病理表现中管腔和实性上皮细胞条索连接成的蝌蚪样结构为其特征性表现,部分患者真皮中上部可见粟丘疹样角质囊肿。Objective: To summarize the clinical and pathological features of genital syringoma, so as to improve the recognition and diagnosis rate of the disease. Methods: The clinical manifestation and histopathological features of 39 patients with genital syringoma diagnosed in our department from January 2010 to September 2019 were retrospectively analyzed. Results:Among the patients, there were 38(97.4%) females and 1(2.6%) male, and the average age was 33.8 years old. The clinical diagnostic rate was only 61.5%, with the most common misdiagnosis of Fox-Fordyce disease. Histopathology showed round or oval cell masses and tubular structure composed of basophilic epithelial cells in the middle and upper dermis, the walls of cavity consisted of two layers of epithelial cells with amorphous eosinophilic substance in cavity. Fifteen cases(38.5%)showed millet papule-like structure, loose horniness in the capsule, and proliferation of fibrous tissues around the tumor mass.Immunohistochemical test showed that all the cases were positive for CK5/6(luminal side of the ducts and keratinous cysts)and CEA(ducts), and negative for S-100 protein. Five(12.8%) cases were scattered positive for CK7. Fifteen cases with milia-like structures were all positive for CK5/6 in the cyst wall, of which 14(93.3%) cases were positive for CEA on the cyst wall, while both keratinous cysts and cell masses were negative for CK7 and S-100 protein. Conclusion: Genital syringoma usually occurs in women, and is most likely to be misdiagnosed as Fox-Fordyce disease. The typical histopathological manifestation is the tadpole-like eccrine ducts composed of lumen and solid epithelial cells. Milia-like keratocysts in the middle and upper dermis can be observed in some patients.

关 键 词：生殖器汗管瘤 粟丘疹 组织病理 

分 类 号：R739.5[医药卫生—肿瘤]