头面部血管肉瘤7例临床组织病理分析并文献回顾  

Angiosarcoma of the face and scalp: 7 cases clinical analysis and literature review

作　　者：杨慧 陈永国 崔静 宋英[1] 常建民[2] YANG Hui;CHEN Yong-guo;CUI Jing(Department of Dermatology and Venerology,Aerospace Center Hospital,Beijing 100049,China)

机构地区：[1]航天中心医院皮肤性病科,北京100049 [2]北京医院皮肤性病科

出　　处：《实用皮肤病学杂志》2021年第5期274-278,共5页Journal of Practical Dermatology

摘　　要：目的探讨头面部血管肉瘤的临床和组织病理特点、诊断及鉴别诊断。方法分析北京医院和航天中心医院2014—2019年7例血管肉瘤患者的临床特点、组织病理检查及免疫组化染色结果,并结合文献复习和讨论。结果7例血管肉瘤患者平均发病年龄68.8岁,临床表现为头面部出现淤斑样皮损,或紫红色结节及溃疡。因临床表现不典型,故误诊率极高。组织病理表现根据分化程度不同而有差异,高分化肿瘤表现为真皮血管样组织增生,低分化则常见细胞异形性,无血管结构。免疫组化标志CD31、CD34有助于明确诊断。该病预后较差,以手术为主的综合治疗为首选治疗方案。结论血管肉瘤临床罕见,临床和组织病理表现不典型,预后较差。发现疑似患者应及时行组织病理及免疫组化检查,尽早明确诊断,并进行综合治疗,改善患者预后。Objective To study the clinical manifestations, histopathological characteristics and differential diagnosis of the face and scalp angiosarcoma. Methods The clinical data of 7 patients with head and neck angiosarcoma who visited Beijing Hospital and Aerospace Center Hospital between 2014 to 2019 were retrospectively analyzed. The medical records of all the 7 patients include clinical manifestations,histopathology, immunohistochemical staining were analyzed and the related literatures were reviewed. Results The average on set age of the 7 patients was 68.8 years old. The common clinical manifestations were bruise-like lesion, erythematous plaque accompanied by nodules, and ulcerations on the face and scalp. Because of the atypical clinical manifestations, the misdiagnosis rate of the disease was high.Pathological examination revealed varing degrees of differentiation and atypia of the tumor cells, as well as obvious proliferation of the endothelial cells, well differentiated ones may show extensive vascular proliferation and infiltration in the dermis, but poorly differentiated ones always present atypical nuclear, and without vascular structures. Immunohistochemical staining(CD31, CD34) is crucial for the diagnosis. Angiosarcoma is a disease with poor prognosis, as the chief component of combined therapy, surgical excision is the cornerstone of the treatment.Conclusion Angiosarcoma is a rare and heterogeneous sarcoma with diverse clinical presentations. When suspected cases are encountered,a biopsy should be taken without delay, and surgery based combined therapy may improve the prognosis of the patients.

关 键 词：血管肉瘤 肿瘤 头面部 临床分析 组织病理 

分 类 号：R732.2[医药卫生—肿瘤]