隆突性皮肤纤维肉瘤28例临床病理分析

Clinicopathological analysis on 28 cases with dermatofibrosarcoma protuberans

目的探讨隆突性皮肤纤维肉瘤(DFSP)的临床病理及免疫组化表达特征,讨论其诊断和鉴别诊断要点,提高对该病的认识。方法收集28例诊断为DFSP的患者,对其临床表现、组织病理学特点、治疗和预后进行回顾性分析。结果28例患者中男17例,女11例[(38.5±14.3)岁],临床表现为皮肤斑块、斑片或结节。组织病理学特征为肿瘤由单一的梭形细胞呈席纹状或车辐状排列,浸润性生长。免疫组化染色肿瘤细胞成分主要表达CD34和Vim。结论 DFSP皮损变化多样,容易误诊,主要依赖组织病理学和免疫组化染色来正确诊断。需与其它梭形细胞肿瘤鉴别。

Objective To investigate the clinicopathological and immunohistochemical features of dermatofibrosarcoma protu- berans （ DFSP） ,and to discuss the diagnosis and differential diagnosis of DFSP in order to raise the awareness of the disease.Methods A retrospective study was performed on 28 patients diagnosed as DFSP.The clinical manifestations, histopathological and immunohistochemical features’treatment and prognosis of DFSP were retrospectively reviewed.Results There were 17 males and 11 females.The average age at onset of DFSP was （38. 5 ± 14.3） years old.Clinical manifestation included skin patches,plaques or nodules.Histological examination revealed that the tumor was composed of uniform infiltrative spindle cells arranged in a storiform or cartwheel pattern.Immunohistochemical staining revealed that tumor cells were positive for CD34 and Vim. Conclusion DFSP has multiplex clinical symptoms and manifestations.Histopathological and immunohistochemical diagnosis is particularly important because it is misdiagnosed easily.DFSP should be differentiated from other spindle cell tumors.