Mani Suresh, Kumar Rajeev, Kakkar Aanchal, Barwad Adarsh, Dheeraj Kondamudi, Sagar Prem, Kumar Rakesh
Department of Head and Neck Surgery, CMC, Vellore, India.
Department of ENT & Head and Neck Surgery, AIIMS, New Delhi, India.
Indian J Surg Oncol. 2023 Mar;14(1):128-136. doi: 10.1007/s13193-022-01636-1. Epub 2022 Sep 1.
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3 months. The duration of recurrence is between 3 and 24 months. The size of the tumor ranges from 7.2 to 10.5 cm. The wide local excision margins range from 2 to 4 cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的皮肤肉瘤,由真皮成纤维细胞发展而来,在真皮和皮下脂肪内扩散。它具有局部侵袭性,切除后局部复发率高,但转移潜能极低。对于复发性肿瘤,手术切除切缘足够是金标准治疗方法,在某些情况下可能需要辅助放疗或化疗。我们对2016年至2021年在我们机构接受治疗的头颈部隆突性皮肤纤维肉瘤患者进行了回顾性分析。我们收集了手术技术、所采用的重建技术、组织病理学特征、辅助治疗及结果的数据。我们治疗了3名头颈部隆突性皮肤纤维肉瘤患者:1例头皮病变,2例脸颊病变。所有3例患者均为复发性肿瘤,其中2例曾在其他地方接受过原发性病变的治疗。1例患者因右侧脸颊良性梭形细胞瘤接受手术,但最终组织病理学检查显示为隆突性皮肤纤维肉瘤,肿瘤在3个月内复发。复发持续时间为3至24个月。肿瘤大小为7.2至10.5厘米。广泛局部切除切缘为2至4厘米。重建方式从刃厚皮片移植到区域皮瓣。切缘不足会增加隆突性皮肤纤维肉瘤局部复发的可能性。