先天性平滑肌错构瘤16例临床及组织病理分析  

Clinical and histopathological features of congenital smooth muscle hamartoma:an analysis of 16 cases

作　　者：陈凤鸣[1] 屈欢欢[1] 张宇伟 王雷[1] 刘玲[1] CHEN Fengming;QU Huanhuan;ZHANG Yuwei;WANG Lei;LIU Ling(Depatement of Dermatology,Xijing Hospital,Fourth Military Medical University,Xi'an 710032,China)

机构地区：[1]第四军医大学西京皮肤医院,陕西西安710032

出　　处：《临床皮肤科杂志》2023年第3期141-145,共5页Journal of Clinical Dermatology

摘　　要：目的:探讨先天性平滑肌错构瘤(CSMH)的临床及组织病理特点。方法:回顾性分析2014年1月-2020年10月于该皮肤医院确诊的16例CSMH患者的临床及组织病理资料。结果:16例患者中女性9例(56.3%),男性7例(43.7%),就诊年龄2~28岁,平均(11±12)岁。其中15例患者(93.7%)出生即有皮损,1例(6.3%)于2岁发病。15例患者(93.7%)表现为单发皮损,1例(6.3%)为多发皮损;皮损发生于面部者11例(68.7%),四肢者3例(18.8%),躯干者2例(12.5%);皮损表现为斑片或斑块者14例(87.5%),表现为群集性丘疹者2例(12.5%);皮损颜色为褐色者11例(68.7%),红色者3例(18.8%),肤色者2例(12.5%);8例患者(50.0%)表现为多毛。皮肤镜下可见病变区域毛发数量增加。皮损组织病理均表现为真皮内无一定走向的成熟平滑肌束,肿瘤细胞胞质呈明显的嗜酸性,细胞核呈雪茄样,两端钝圆。免疫组化示肿瘤细胞表达平滑肌肌动蛋白(SMA)及结蛋白(desmin)。结论:CSMH多出生即有,单发多见,可发生于面部、四肢和躯干,主要表现为褐色斑片,少见局部毛发数量增加。该病的诊断主要依靠皮损组织病理检查,皮肤镜可用于辅助诊断。Objective:To investigate the clinical and histopathological features of congenital smooth muscle hamartoma(CSMH).Methods:The clinical and histopathological data of 16 patients with CSMH diagnosed in the Department of Dermatology of Xijing Hospital from January 2014 to October 2020 were retrospectively analyzed.Results:Among the 16 patients,9 were female(56.3%)and 7 were male(43.7%).The patients aged 2 years to 28 years,with an average of 11±12 years.Among them,15 patients(93.7%)had skin lesions at birth,and 1 patient(6.3%)had onset at 2 years old.Fifteen patients(93.7%)had a single lesion and 1 patient(6.3%)had multiple lesions.CSMH lesions were predominantly located on face(11 cases,68.7%),followed by extremities(3 cases,18.8%)and trunk(2 cases,12.5%).Fourteen cases(87.5%)presented patches or plaques,and 2 cases(12.5%)presented clusters of papules.The lesions were brown in 11 cases(68.7%),red in 3 cases(18.8%),and skin color in 2 cases(12.5%).Hypertrichosis was found in 8 cases(50.0%).Dermoscopic findings showed the increase of hair density.The histopathological examination of CSMH showed disseminated proliferation of mature smooth muscle cells of a central cigar-shaped nucleus and fibrillary and eosinophilic cytoplasm in the dermis.Immunohistochemistry showed the tumor cells were positive for smooth muscle actin(SMA)and desmin.Conclusion:CSMH usually presents at birth as a solitary brown patch covered with overlying hypertrichosis on the face,limbs,and trunk.Dermoscopy and histopathological examination are helpful to establish a diagnosis.

关 键 词：平滑肌错构瘤 先天性 组织病理 皮肤镜 

分 类 号：R739.5[医药卫生—肿瘤]