Bowne W B, Antonescu C R, Leung D H, Katz S C, Hawkins W G, Woodruff J M, Brennan M F, Lewis J J
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Cancer. 2000 Jun 15;88(12):2711-20.
Despite optimal surgical therapy for patients with dermatofibrosarcoma protuberans (DFSP), some patients still continue to develop local recurrence. The authors' objective was to identify and analyze clinicopathologic factors for disease free survival in a large group of patients who were followed prospectively at a single institution.
Prospectively collected data and pathology slides were available for review from 159 patients with primary or recurrent DFSP who underwent treatment between July 1950 and July 1998. The study group was comprised of patients with either the "classic" form of DFSP or the fibrosarcomatous "high grade" variant of DFSP (FS-DFSP). Patient, tumor, pathologic, and treatment factors were analyzed using the log rank test for univariate influence and Cox regression analysis for multivariate influence. Local recurrence free survival was determined by the Kaplan-Meier actuarial method.
Of the 159 patients who comprised the current study group, 134 (84%) had the classic form of DFSP. The FS-DFSP variant was found in the remaining 25 patients (16%). The overall 5-year local recurrence free survival rate was 75%, with a median follow-up of 4. 75 years. The 5-year recurrence free survival rate for each group was 81% and 28%, respectively. On univariate analysis, age > 50 years, very close (< 1 mm) to positive microscopic margins, FS-DFSP variant, high mitotic rate, and increased cellularity were unfavorable prognostic factors. Multivariate analysis determined very close (< 1 mm) to positive microscopic margins and FS-DFSP variant to be independent adverse prognostic factors. For the 34 patients who developed a recurrence after surgical resection (21%), the median time to local recurrence was 32 months. Of the patients in this group, two died from metastatic disease.
The prognosis after surgical resection with negative and sometimes positive microscopic margins for patients with DFSP is very good. However, increased age, high mitotic index, and increased cellularity are predictors of poor clinical outcome. The FS-DFSP variant represents a much more aggressive tumor with metastatic potential. Patients who are treated with curative intent for FS-DFSP should undergo aggressive attempts at complete surgical resection. Patients with recurrent classic DFSP without evidence of adverse prognostic features may benefit from conservative management, especially in the setting of potentially unresectable disease.
尽管对隆突性皮肤纤维肉瘤（DFSP）患者采用了最佳手术治疗，但仍有一些患者会出现局部复发。作者的目的是在一个单一机构前瞻性随访的一大组患者中，识别并分析无病生存的临床病理因素。
前瞻性收集了1950年7月至1998年7月期间接受治疗的159例原发性或复发性DFSP患者的数据和病理切片以供回顾。研究组包括“经典”型DFSP患者或DFSP的纤维肉瘤样“高级别”变体（FS-DFSP）患者。使用对数秩检验分析患者、肿瘤、病理和治疗因素的单变量影响，并使用Cox回归分析多变量影响。局部无复发生存率通过Kaplan-Meier精算方法确定。
在构成本研究组的159例患者中，134例（84%）为经典型DFSP。其余25例（16%）为FS-DFSP变体。总体5年局部无复发生存率为75%，中位随访时间为4.75年。每组的5年无复发生存率分别为81%和28%。单变量分析显示，年龄>50岁、显微镜下切缘非常接近（<1mm）阳性、FS-DFSP变体、高有丝分裂率和细胞增多是不良预后因素。多变量分析确定显微镜下切缘非常接近（<1mm）阳性和FS-DFSP变体是独立的不良预后因素。在手术切除后复发的34例患者（21%）中，局部复发的中位时间为32个月。该组患者中有2例死于转移性疾病。
对于DFSP患者，手术切除后显微镜下切缘阴性甚至阳性时的预后非常好。然而，年龄增加、有丝分裂指数高和细胞增多是临床结局不良的预测因素。FS-DFSP变体代表一种更具侵袭性且有转移潜能的肿瘤。接受FS-DFSP根治性治疗的患者应积极尝试进行完整的手术切除。复发性经典DFSP且无不良预后特征证据的患者可能从保守治疗中获益，尤其是在可能无法切除的疾病情况下。
