隆突性皮肤纤维肉瘤35例临床分析  

Clinical Analysis on 35 Cases of Dermatofibrosarcoma Potuberans

作　　者：孙凯亮 陈辉[2] 李玉梅 周春红 万苗坚[4] 李华 吴涛 

机构地区：[1]珠海市慢性病防治中心,广东珠海519070 [2]广州医科大学附属第三医院病理科,广东广州510150 [3]珠海市第二人民医院病理科,广东珠海519000 [4]中山大学附属第三医院皮肤科,广东广州510630 [5]广州市金域医学检验中心,广东广州510330

出　　处：《皮肤性病诊疗学杂志》2016年第6期367-371,375,共6页Journal of Diagnosis and Therapy on Dermato-venereology

摘　　要：目的:探讨隆突性皮肤纤维肉瘤(DFSP)的临床特点、超声表现、手术治疗以及病理诊断和鉴别诊断要点。方法:回顾性分析35例DFSP患者的临床表现、超声特点、组织病理学特点、治疗和预后。结果:35例患者中男性22例,女性13例;平均年龄(35.4±12.4)岁。临床典型表现为皮肤斑块、斑片或结节,未见溃疡。躯干最为多见。超声:肿物位于皮下,肿物边界清楚,边缘尚规则,内呈分叶状,瘤体内有低回声或混合性回声,其内可见稀疏或较丰富的血流信号。组织病理学特征为一致性梭形肿瘤细胞呈席纹状或车辐状排列,浸润性生长,部分区域肿瘤细胞异型性明显,似纤维肉瘤样改变,肿瘤细胞表达Vimentin。本组病例MMS手术者1例术后1年复发,2例术后3年复发。结论:DFSP形态多样,临床容易误诊,B超检查对诊断、判断肿物边界以及手术治疗有一定的指导意义,组织病理及免疫组化检查可进一步确诊。MMS对本病治疗有很大优势。Objective： To discuss the clinical characteristics, sonographic fndings, surgical treatment and pathological diagnosis and differential diagnosis of dermatofibrosarcoma protuberans （DFSP）. Methods： 35 cases of DFSP were analyzed retrospectively. The clinical features, ultrasonic features, histopathological features, treatment and prognosis were retrospectively analyzed. Results： There were 22 males and 13 females, the average age of the patients were （35.4 ±12.4） years old. Typical clinical manifestation included skin patches, plaques or nodules, and without ulcer. The involved area in most patients is the trunk. Histopathological characteristics for the consistency of spindle tumor cells showed storiform or radial wheel-like arrangement, infiltrative growth, focal prominent atypic tumor cells, similar to changes in fiber sarcomatoid tumor. Tumor cells expressed vimentin. There were one case of recurrence in this group of patients with MMS after 1 year, and 2 cases after 3 years. Conclusion： DFSP has multiplex clinical manifestations and was easily to be misdiagnosed. It can be further confirmed by tissue pathology and im- mune group examination. MMS is the first choice in its treatment.

关 键 词：隆突性皮肤纤维肉瘤 临床表现 超声表现 MMS手术 病理特点 

分 类 号：R739.5[医药卫生—肿瘤]