隆突性皮肤纤维肉瘤进展为纤维肉瘤亚型高危因素分析

Analysis of high risk factors for progression of dermatofibrosarcoma protuberans to fibrosarcomatous subtype dermatofibrosarcoma protuberans

目的对比典型隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)及纤维肉瘤亚型(fibrosarcomatous subtype dermatofibrosarcoma protuberans,FS-DFSP)的差异,总结不同特征,并寻找DFSP进展为FS-DFSP病变的高危因素。方法收集2014年2月至2021年8月香港大学深圳医院诊断为DFSP和FS-DFSP的34例患者,根据诊断将患者分为典型DFSP组(24例)及FS-DFSP组(10例),收集患者基本信息并随访,对比两组的发病时间、肿瘤大小、位置、病灶免疫组化、术前治疗等差异,并进行Kaplan-Meier生存分析。结果FS-DFSP组的肿瘤直径(59.80±31.48)mm大于典型DFSP组(40.71±19.67)mm,其差异具有统计学意义(P<0.05);FS-DFSP组平均发病时间7.7年,典型组DFSP平均5.2年,差异无统计学意义(P>0.05);FS-DFSP组Ki67表达率(25.60±13.43)%高于典型DFSP组(13.94±9.96)%,FS-DFSP组SMA阳性率高于典型DFSP组,其差异具有统计学意义(P<0.05);FS-DFSP组有70%来源于单纯切除后复发高于DFSP组的25%,其差异均具有统计学意义(P<0.05)。结论FS-DFSP更具强侵袭性,转移复发率较高,与典型DFSP具有明显不同的临床特征表现;单纯切除是典型DFSP进展为FS-DFSP的高危因素,建议尽早Ⅱ期扩大切除,减少肿瘤进展的风险。

Objective To compare the differences between typical dermatofibrosarcoma protuberans(DFSP)and fibrosarcomatous subtype dermatofibrosarcoma protuberans(FS-DFSP).To summarize different characteristics and to investigate the risk factors of DFSP progressing to FS-DFSP.Methods From February 2014 to August 2021,34 patients diagnosed with DFSP and FS-DFSP were collected.All patients were divided into the typical DFSP group(24 cases)and the FS-DFSP group(10 cases).Basic information of all patients was collected.The follow-up was conducted to compare the differences in time of onset,size of tumor,location of lesion,immunohistochemistry and preoperative treatment between both groups.The Kaplan-Meier method was used for survival analysis.Results The tumor diameter in the FS-DFSP group was(59.80±31.48)mm,which was higher than(40.71±19.67)mm in the typical DFSP group.The difference was statistically significant(P<0.05).The average duration of onset was 7.7 years in the FS-DFSP group and 5.2 years in the typical DFSP group.The difference was not statistically significant(P>0.05).The expression of Ki67 in the FS-DFSP group was(25.60±13.43)%,which was higher than(13.94±9.96)%in the typical DFSP group.The positive rate of SMA in the FS-DFSP group was higher than that in the typical DFSP group,the difference was statistically significant(P<0.05).In the FS-DFSP group,70%of patients had recurrence after simple resection,which was higher than 25%in the typical DFSP group.The difference was statistically significant(P<0.05).Conclusion FS-DFSP have high invasiveness and recurrence rate of metastasis,which is obviously different from typical DFSP in clinical manifestations.Simple resection is a high risk factor for progression of typical DFSP to FS-DFSP.It is recommended to expand the resection in stageⅡas early as possible to reduce the risk of tumor progression.