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头颈部隆突性皮肤纤维肉瘤:广泛切除与重建病例系列并文献综述

Head and neck dermatofibrosarcoma protuberans: Case series of extensive resections and reconstructions with literature review.

作者信息

Kedous Skander, Amri Ameni, Methnani Alia, Fertani Yasmine, Gallas Amira, Braham Rim, Dhaha Mohamed, Jbali Souhail, Dhembri Sawssen

机构信息

ENT Head & Neck Surgery Department, Salah Azaiz Institute, Tunis, Tunisia.

Plastic and Reconstructive Surgery Department, Salah Azaiz Institute, Tunis, Tunisia.

出版信息

F1000Res. 2025 May 12;14:294. doi: 10.12688/f1000research.162699.2. eCollection 2025.

Abstract

Dermatofibrosarcoma protuberans is a rare, locally aggressive soft-tissue sarcoma. Head and neck involvement accounts for only 10-15% of cases. Achieving clear margins in this region is challenging owing to anatomical constraints. Such cases often require extensive resection and complex reconstructions. This study presents a case series of extensive dermatofibrosarcoma resections in the head and neck, focusing on the surgical margins, reconstruction strategies, recurrence rates, and adjuvant therapy. We report four cases of head and neck dermatofibrosarcoma involving the cheek and scalp. Surgery included wide local excision with margins of 3-5 cm, which was confirmed intraoperatively by frozen section analysis. Reconstruction involved free flaps, local flaps, and healing with secondary intention. The choice depends on the defect size and location. Adjuvant radiotherapy was administered to selected cases. All patients achieved negative margins. One patient developed flap necrosis that required revision surgery. No local recurrence was observed during the follow-up (1-7 years). Head and neck dermatofibrosarcoma justifies aggressive surgical resection to achieve clear margins, which is the key to reducing the risk of recurrence. Free flaps and local reconstruction techniques ensure good functional and aesthetic outcomes. Adjuvant radiotherapy is indicated in patients with close margins or deep invasion. Long-term follow-up is essential owing to its high recurrence potential.

摘要

隆突性皮肤纤维肉瘤是一种罕见的、局部侵袭性软组织肉瘤。头颈部受累仅占病例的10 - 15%。由于解剖学限制,在该区域实现切缘阴性具有挑战性。此类病例通常需要广泛切除和复杂重建。本研究呈现了一组头颈部广泛切除隆突性皮肤纤维肉瘤的病例系列,重点关注手术切缘、重建策略、复发率和辅助治疗。我们报告了4例累及面颊和头皮的头颈部隆突性皮肤纤维肉瘤病例。手术包括切缘为3 - 5 cm的广泛局部切除,术中通过冰冻切片分析确认切缘情况。重建方式包括游离皮瓣、局部皮瓣和二期愈合,具体选择取决于缺损大小和位置。部分病例接受了辅助放疗。所有患者均实现了切缘阴性。1例患者发生皮瓣坏死,需要进行修复手术。随访期间(1 - 7年)未观察到局部复发。头颈部隆突性皮肤纤维肉瘤需要积极的手术切除以实现切缘阴性,这是降低复发风险的关键。游离皮瓣和局部重建技术可确保良好的功能和美学效果。切缘阳性或深部浸润的患者需要进行辅助放疗。鉴于其高复发潜能,长期随访至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ff97/12136165/f294490aa1b7/f1000research-14-181850-g0000.jpg

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