隆突性皮肤纤维肉瘤36例临床分析  

An analysis of dermatofibrosarcoma protuberans in 36 patients

作　　者：李艳[1] 杨子良[1] 宋琳毅[1] 周乃慧[1] LI Yan;YANG Ziliang;SONG Linyi;ZHOU Naihui(Department of Dermatology,the First Afiliated Hospital of Soochow University,Suzhou 215006,China)

机构地区：[1]苏州大学附属第一医院皮肤科,江苏苏州215006

出　　处：《临床皮肤科杂志》2023年第3期131-134,共4页Journal of Clinical Dermatology

摘　　要：目的:总结隆突性皮肤纤维肉瘤(DFSP)的临床表现、影像学表现及组织病理特点。方法:回顾性分析1988年9月-2020年12月就诊于苏州大学附属第一医院的36例DFSP患者的临床及组织病理资料。结果:36例DFSP患者中,男女比例1.6:1;平均发病年龄(36.8±11.4)岁。皮损以躯干最为多见[25例(69.4%)],其次为四肢[7例(19.4%)]。超声检查示肿瘤位于真皮及皮下层,呈不均匀低回声,边界清楚,肿瘤内具有较丰富的血流信号。磁共振成像(MRI)平扫示T1WI呈等或低信号,T2WI呈高信号,增强后可见明显均匀强化。36例患者组织病理分型均为经典型,临床诊断与病理诊断符合率为16.7%,免疫组化示CD34和vimentin表达阳性,S-100蛋白和CD68表达阴性。该组患者术后复发率为27.6%。结论:DFSP多见于中年男性,病程缓慢,临床误诊率高。超声及MRI等影像学检查可为疾病的诊断、手术方式及范围提供参考。该病复发率高,手术应局部扩大切除,尤其需注意切除的范围和深度,加强长期临床随访。Objective:To summarize the clinical,imaging and histopathological features of dermatofibrosarcoma protuberans(DFSP).Methods:We retrospectively analyzed the clinical and pathological data of 36 patients from the First Affiliated Hospital of Soochow University from September 1988 to December 2020.Results:Of the 36 cases,the male/female ratio was 1.6:1.The mean age of onset was 36.8±11.4 years.Twenty-five cases(69.4%)showed a DFSP in the trunk,followed by 7 cases(19.4%)in the limbs.Ultrasonographic findings showed that the tumor was located in the dermis and subcutaneous layer,presenting un-even hypoecho,clear boundaries,and abundant blood flow signals.Plain MRI showed isointense or lowintense signals on TIWI and hyperintense signals on T2WI.After intravenous injection,remarkably homogeneous enhancement was observed.All the 36 patients were pathologically classified as typical dermatofibrosarcoma protuberans,and the accordance rate between clinical di-agnosis and pathological diagnosis was 16.7%.Immunohistochemical results showed positive CD34 and vimentin,and negative S-100 and CD68.The postoperative recurrence rate was 27.6%.Conclusion:DFSP is more common in middle aged men,with a slow progression and a high rate of clinical misdiagnosis.Imaging tools like ultrasound and MRI can provide reference for diagnosis and surgical treatment.The recurrence rate is high,and extensive local resection at a certain depth should be performed.Long-term clinical follow-up should be considered.

关 键 词：隆突性皮肤纤维肉瘤 临床 组织病理 

分 类 号：R739.5[医药卫生—肿瘤]