假肌源性血管内皮瘤2例并文献复习  

Pseudomyogenic hemangioendothelioma:report of two cases and literature review

作　　者：田翠翠 宋昊 姜祎群 孙建方 陈浩 TIAN Cuicui;SONG Hao;JIANG Yiqun;SUN Jianfang;CHEN Hao(Department of Pathology,Institute of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)

机构地区：[1]中国医学科学院、北京协和医学院皮肤病医院病理科,江苏南京210042

出　　处：《临床皮肤科杂志》2025年第2期100-104,共5页Journal of Clinical Dermatology

基　　金：中国医学科学院医学与健康科技创新工程临床转化专项(2023-I2M-C&T-B-110)。

摘　　要：报告2例假肌源性血管内皮瘤(PMH)。例1.患者女,38岁,左手背红色丘疹6年。皮肤科检查:左手背蚕豆大红色结节,质硬,轻度压痛。皮损组织病理检查:真皮内境界不清的肿瘤团块,中央可见灶状坏死区,肿瘤细胞呈上皮样,细胞质丰富,核仁明显,外形不规则,可见核分裂象,肿瘤团块周围部分细胞呈栅栏状排列。免疫组化:细胞角蛋白(CK)、ETS相关基因蛋白(ERG)及CD31均(+);增殖核抗原(Ki-67)(10%+);CD34、P63、上皮膜抗原(EMA)及S-100蛋白均(-)。诊断:PMH。例2.患者男,69岁,左足底紫红色丘疹伴疼痛5个月。皮肤科检查:左足底散在紫红色丘疹,表面结痂。皮损组织病理检查:表皮角化过度,棘层肥厚,真皮中下部可见境界不清的肿瘤细胞团块,呈束状、漩涡状排列,肿瘤细胞呈梭形,部分呈上皮样,细胞质丰富、红染,细胞核轻度异形,偶见核分裂象。肿瘤间质散在炎性细胞浸润。免疫组化:CK、波形蛋白及ERG均(+);CD31及平滑肌肌动蛋白(SMA)(部分+);CD34、D2-40、S-100蛋白、EMA及结蛋白均(-);Ki-67(3%+)。诊断:PMH。Two cases of pseudomyogenic hemangioendothelioma(PMH)are reported.Case 1.A 38-year-old female patient who presented with a red papule on the dorsum of her left hand for six years.Physical examination revealed a fava bean-sized red nodule on the dorsum of the left hand,which was hard and slightly tender.Histopathological examination of the lesion showed an unclear boundary of tumor masses in the dermis with focal necrotic area in the center.The tumor cells were epithelioid with abundant cytoplasm,distinct nucleolus and abnormal mitotic figures.Some cells around the tumor masses were arranged in a palisade pattern.Immunohistochemical test showed cytokeratin(CK),ETS-related gene protein(ERG)and CD31 were all positive.Ki-67 was 10%positive.CD34,P63,epithelial membrane antigen(EMA)and S-100 protein were all negative.A diagnosis of PMH was made.Case 2.A 69-year-old male who presented with purplish papules on the left sole with pain for five months.Physical examination revealed a purplish scattered papules with scabs on the left sole.Histopathological examination of the lesions showed hyperkeratosis and acanthosis.Indistinct clusters of tumor cells arranged in bundles,and swirls were seen in the middle and lower dermis.The tumor cells had a fusiform shape,and some of them were epithelial cells.The cytoplasm of tumor cells was rich and red stained.The nuclei of tumor cells were mildly deformed,and the mitotic images were occasionally seen.Scattered inflammatory cell infiltration was seen in the tumor stroma.Immunohistochemical test showed CK,vimentin and ERG were positive.CD31 and smooth muscle actin(SMA)were partially positive.CD34,D2-40,S-100 protein,EMA and desmin were all negative.Ki-67 was 3%positive.A diagnosis of PMH was made.

关 键 词：假肌源性血管内皮瘤 上皮样肉瘤样血管内皮瘤 软组织肿瘤 

分 类 号：R739.5[医药卫生—肿瘤]