Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Dermatologic Oncology, National Cancer Center Hospital East, Kashiwa, Japan.
J Dermatol. 2024 Sep;51(9):1225-1232. doi: 10.1111/1346-8138.17280. Epub 2024 May 22.
Cutaneous dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor characterized by a high risk of local recurrence but a low risk of metastasis. Wide local excision (WLE) has been an important treatment option, but its clinical outcomes and safety have not been thoroughly evaluated in previous reports. The aim of this study was to determine appropriate surgical margins (deep and lateral) and prognostic factors associated with recurrence-free survival (RFS) of DFSP. A database collected by two dermatology departments in Japan was retrospectively reviewed to identify 116 patients with DFSP who underwent complete resection with WLE between 1994 and 2021. Sixty-one men (53%) and 55 women (47%) were included in our cohort. The primary sites of DFSP were as follows: 11 head and neck (9%); seven face (7%); 12 upper extremities (10%); 20 lower extremities (17%); and 66 trunk (57%). There were 103 cases (89%) of primary DFSP and 13 cases (11%) of recurrent DFSP. Total 10-year RFS was 96.6%. There were significant differences in RFS by tumor size (median size: 3 cm), disease status (primary versus recurrent DFSP), and fibrosarcomatous change (positive versus negative) (all p < 0.05). Two patients (1.7%) with buccal or head lesions had positive deep margins. In all cases, the lateral margin was negative at the postoperative evaluation. Tumor size, disease status, and fibrosarcomatous change are important risk factors for recurrence. Both face and head-neck lesions were more likely to have positive deep margins than other anatomic areas in DFSP. Although this study was limited by its retrospective design, a narrow 2-cm lateral margin is especially considered for low-risk patients.
隆突性皮肤纤维肉瘤(DFSP)是一种纤维组织细胞肿瘤,具有较高的局部复发风险,但转移风险较低。广泛局部切除术(WLE)一直是一种重要的治疗选择,但之前的报告并未彻底评估其临床结果和安全性。本研究旨在确定 DFSP 无复发生存(RFS)相关的适当手术切缘(深度和侧面)和预后因素。回顾性分析日本两个皮肤科部门收集的数据库,确定了 1994 年至 2021 年间接受 WLE 完全切除的 116 例 DFSP 患者。我们的队列包括 61 名男性(53%)和 55 名女性(47%)。DFSP 的主要部位如下:11 例头颈部(9%);7 例面部(7%);12 例上肢(10%);20 例下肢(17%);66 例躯干(57%)。103 例(89%)为原发性 DFSP,13 例(11%)为复发性 DFSP。10 年 RFS 为 96.6%。肿瘤大小(中位大小:3cm)、疾病状态(原发性与复发性 DFSP)和纤维肉瘤样变(阳性与阴性)均显著影响 RFS(均 p<0.05)。2 例颊部或头部病变患者的深部切缘阳性。所有病例术后评估均为侧面切缘阴性。肿瘤大小、疾病状态和纤维肉瘤样变是复发的重要危险因素。面部和头颈部病变比其他解剖区域更有可能出现深部切缘阳性。尽管本研究受到回顾性设计的限制,但对于低危患者,尤其考虑采用 2cm 窄的侧面切缘。