儿童隆突性皮肤纤维肉瘤临床病理特点与外科治疗结果单中心研究

目的 研究儿童隆突性皮肤纤维肉瘤(pediatric dermatofibrosarcoma protuberans, PDFSP)的临床与病理特点、诊断与外科治疗现状及预后.方法 回顾性研究北京大学肿瘤医院骨与软组织肿瘤科2007年7月至2015年7月间收治的8例PDFSP患儿的临床、病理资料与外科治疗结果.结果 本组男5例,女3例;年龄8～18岁,中位年龄13.5岁;肿瘤大小为0.5～6.5 cm,平均2.8cm,好发部位(躯干与四肢近端占5/8)与病理特点(均为经典型)类似成年患者,未见肢端表现或特殊病理亚型.确诊时间明显滞后于发病时间,达3～96个月,平均27.8个月.8例患儿20次病理诊断的总确诊率为50％(10/20),诊前获CD34免疫组织化学染色阳性率为38.9％(7/18),两种比率呈线性相关趋势,但限于样本较少,差异无统计学意义(Spearman线性相关分析,P=0.333,r=0.866).所有确诊例次中诊前获CD34阳性比率显著高于未确诊例次,差异有统计学意义(7/8 vs 0/10,Fisher test,P=0.020).8例患儿均行扩大切除治疗,5例为原发手术(1例肿瘤扩大切除,4例瘤床补充扩大切除),3例为复发手术(均为肿瘤扩大切除).随访时间6～96个月,中位随访时间48.5个月,术后复发率为12.5％(1/8),未见远处转移或死亡病例.局部切除是PDFSP首诊的主要治疗方法(7/8),局部切除后行规范性扩大切除4例均无复发,未行规范性扩大切除3例全部复发,两项比较,差异有统计学意义(0/4 vs.3/3,Fisher test,P=0.20).术中送检冰冻病理切缘,有助于扩大切除的有效性.结论 作为中国内地首个PDFSP病例系列,显示与文献类似的某些临床特征与较好预后.CD34阳性在PDFSP诊断中具有关键作用.肿瘤局部切除后,及时纠正诊断并做规范性补充扩大切除,是保证患儿预后的关键.亟需增加病理诊断准确性与手术治疗规范性,以期改善预后.

Clinicopathological characteristics and surgical outcomes of pediatric dermatofibrosarcoma protuberans: a study of single-institutional series

Objective To explore the clinicopathological characteristics,diagnosis,treatment and prognosis of pediatric dermatofibrosarcoma protuberans (PDFSP).Methods A total of 8 cases of PDFSP were treated from July 2007 to July 2015 at Department of Orthopedic Oncology,Peking University Cancer Hospital.Retrospective analyses were performed for their clinicopathological data and surgical outcomes.Results There were 5 males and 3 females with a median age of 13.5 (8-18) years.And the mean tumor size was 2.8 (0.5-6.5) cm.The frequent locations (5/8 at trunk & proximal extremity) and pathological features (all as conventional variants) were similar to those of adults.No case was located at acral sites and there was not any special pathological variant.The delay duration between presentation and diagnosis was 27.8 (3-96) months.The overall correct diagnostic rate of all practices was 50％ (10/20).And the overall positive rate of CD34 immumohistochemieal staining was 38.9％ (7/18).A correlative trend existed between these two rates.However,no significance existed due to limited cases (Spearman's correlation analysis,P =0.333,r =0.866).The positive rate of CD34 by a correct diagnosis was significantly higher than that by others (7/8 vs 0/10,Fisher's test,P =0.02).Among them,five wide local excisions (WLE) were performed on primary diseases for tumor (n =1),tumor bed (n =4) and three on recurrent diseases for tumor (n =3).During a median follow-up period of 48.5 (6-96) months,the recurrent rate was 12.5％ (1/8).Neither metastasis nor mortality was found.Most (7/8) of initial therapies were local excisions.All salvaged with standardized WLE (n =4) had no recurrence while those non-salvaged with standardized WLE (n =3) were all recurrent (0/4 vs 3/3,Fisher's test,P =0.20).Intra-operative frozen margins were beneficial.Conclusions As the first-ever reported series of PDFSP in mainland of China,it shares some clinical features and favorable outcomes with the literature.For a correct diagnosis,it is essential to attain positive result of CD34.To ensure favorable prognosis,timely correction of misdiagnosis and salvaging by standardized WLE are necessary after local excision.We should pay more attention to improving the rate of correct diagnosis and standardizing WLE.