结节性纤维肉瘤是一种罕见的、生长缓慢的软组织恶性肿瘤，起源于真皮，其特征为浸润性生长模式和明显的局部复发倾向。必须完成完整的手术切除并达到病理边缘清除，以降低肿瘤复发的风险。由此产生的缺陷通常需要进行广泛的重建手术。头皮结节性纤维肉瘤面临特殊的挑战，由于它接近面部和大脑。本研究旨在评估治疗选项，并基于多中心病例系列和系统文献综述提出头皮结节性纤维肉瘤管理的算法。对过去20年内诊断的11例头皮结节性纤维肉瘤患者的人口统计学数据、病理肿瘤特征和手术治疗（切除和重建）进行了回顾性多中心图表分析。此外，通过对文献进行系统的优先报道系统综述和Meta分析，筛选出另外42例患者（44例病例），共计30例头皮结节性纤维肉瘤为原发性病例，20例为复发性病例（5例缺失数据）。中位数肿瘤大小为24平方厘米（四分位差7.8-64），中位数缺陷大小为55.8平方厘米（四分位差48-112）。复发性头皮结节性纤维肉瘤更常与深层组织的浸润相关，并需要更广泛的肿瘤切除来达到阴性边缘。在采用外周和深层正面边缘评估管理亚组中，未观察到复发。大多数患者需要进行局部（41.8％）或游离皮瓣（27.8％）重建手术。尽可能地采用外周和深层正面边缘评估技术对头皮结节性纤维肉瘤进行切除，因为它们提供更优越的肿瘤学安全性同时保留未受累的组织。具有局部进展和复发性头皮结节性纤维肉瘤的患者通常需要多学科治疗，包括神经外科、放疗和微型血管重建手术，并应转诊至专业中心。copyright © 2023 Elsevier Inc. All rights reserved.

Dermatofibrosarcoma protuberans is a rare, slow-growing soft-tissue malignancy originating in the dermis that is characterized by an infiltrating growth pattern with a marked tendency of local recurrence. Complete surgical resection with pathological margin clearance must be achieved to reduce the risk of tumor recurrence. Resulting defects often require extensive reconstructive procedures. Dermatofibrosarcoma protuberans of the scalp poses particular challenges owing to the proximity to the face and brain. This study aims to evaluate treatment options and proposes an algorithm for management of scalp dermatofibrosarcoma protuberans based on a multicentric case series and systematic review of the literature.A retrospective multicentric chart analysis of 11 patients with scalp dermatofibrosarcoma protuberans who presented within the last 20 years was performed regarding demographic data, pathological tumor characteristics, and surgical management (resection and reconstruction). Additionally, a further 42 patients (44 cases) were identified through a systematic Preferred Reporting Systems for Systematic Reviews and Meta-Analysis-based review of the literature searching the Medline and Embase databases.In total, 30 cases were classified as primary and 20 cases as recurring scalp dermatofibrosarcoma protuberans (data from 5 cases were missing). The median tumor size was 24 cm2 (interquartile range 7.8-64), and the median defect size was 55.8 cm2 (interquartile range 48-112). Recurring scalp dermatofibrosarcoma protuberans was more often associated with invasion of deeper layers and required more extensive tumor resection to achieve negative margins. Within the subgroup that was managed with peripheral and deep en face margin assessment, no recurrence was observed. Most patients required local (41. 8%) or free flap (27.8%) reconstruction after dermatofibrosarcoma protuberans resection.Whenever possible, peripheral and deep en face margin assessment-based techniques should be preferred for resection of scalp dermatofibrosarcoma protuberans because they provide superior oncological safety while preserving uninvolved tissue. Patients with locally advanced and recurring scalp dermatofibrosarcoma protuberans often require multidisciplinary treatment including neurosurgery, radiotherapy, and microvascular reconstructive surgery and should be referred to a specialized center.Copyright © 2023 Elsevier Inc. All rights reserved.