Sarcoma Unit, Department of Surgery, Royal Marsden Hospital, London, United Kingdom.
Sarcoma Unit, Department of Surgical Oncology, Netherlands Cancer Institute-Antoni van Leeuwenhoek, Amsterdam, The Netherlands.
Cancer. 2019 Mar 1;125(5):735-741. doi: 10.1002/cncr.31924. Epub 2019 Jan 15.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma for which clinical examination up to 10 years is recommended. The objective of this study was to identify prognostic factors for recurrences and metastases that can be used to evaluate the validity of follow-up schedules after treatment for DFSP.
Patients with DFSP who received treatment between 1991 and 2016 at 3 tertiary centers were included. Cox regression analyses were conducted to identify variables associated with the primary endpoints.
In total 357 patients were included, with a median age of 38 years (age range, 2-87 years) and a median follow-up of 60 months (interquartile range, 24-115 months). Eighty-one patients developed recurrent disease (22.7%), and the median time to recurrence was 55.5 months (interquartile range, 20-90 months). Of these, 50 tumors (61.7%) were identified by patient self-examination, whereas 3 recurrences (3.7%) were identified at clinical surveillance. For the remaining 28 tumors, no information was available on how the recurrences were identified (34.6%). Fibrosarcomatous change (hazard ratio, 21.865; P < .001), and positive resection margins (hazard ratio, 14.645; P < .001), were independent prognostic factors for recurrence. Metastases occurred in 4 patients (1.1%). All tumors were identified by imaging after patients presented with symptomatic metastases. Fibrosarcomatous change (P < .001) and tumor size >5 cm (P = .014) were associated with the development of metastases.
Disease recurrence after resection of DFSP remains a significant issue, whereas metastases are uncommon. The majority of recurrences are identified by patient self-examination. Consideration should be given to individualized follow-up schedules based on risk factors for recurrences and metastases.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的软组织肉瘤，建议临床检查随访 10 年。本研究的目的是确定与复发和转移相关的预后因素，这些因素可用于评估 DFSP 治疗后随访计划的有效性。
纳入了 3 个三级中心在 1991 年至 2016 年间治疗的 DFSP 患者。采用 Cox 回归分析确定与主要终点相关的变量。
共纳入 357 例患者，中位年龄为 38 岁（年龄范围：2-87 岁），中位随访时间为 60 个月（四分位距：24-115 个月）。81 例患者发生复发病例（22.7%），中位复发时间为 55.5 个月（四分位距：20-90 个月）。其中，50 例肿瘤（61.7%）由患者自检发现，3 例复发（3.7%）由临床监测发现。对于其余 28 例肿瘤，复发如何被发现的信息不详（34.6%）。纤维肉瘤样变（风险比，21.865；P<0.001）和阳性切缘（风险比，14.645；P<0.001）是复发的独立预后因素。4 例患者（1.1%）发生转移。所有肿瘤均在患者出现症状性转移后通过影像学发现。纤维肉瘤样变（P<0.001）和肿瘤大小>5cm（P=0.014）与转移的发生有关。
DFSP 切除后疾病复发仍然是一个重大问题，而转移则较为罕见。大多数复发是由患者自检发现的。应根据复发和转移的危险因素制定个体化的随访计划。
