Martinez Carlos, McDougal Elizabeth, Gilstrap Jarom, Friedman Harold I, Rivera Mariangela
Prisma Health/University of South Carolina School of Medicine, Department of Surgery, Division of Plastic & Reconstructive Surgery, 2 Medical Park Rd. Suite 302, Columbia, SC 29203, United States.
Prisma Health/University of South Carolina School of Medicine, Department of Surgery, Division of Plastic & Reconstructive Surgery, 2 Medical Park Rd. Suite 302, Columbia, SC 29203, United States.
Int J Surg Case Rep. 2020;76:98-102. doi: 10.1016/j.ijscr.2020.09.172. Epub 2020 Sep 28.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally invasive soft tissue tumor with a high rate of recurrence after surgical intervention. DFSP primarily occurs on the trunk and proximal extremities, with reported 10-15% occurring in the head and neck region. DFSP presenting within the oral cavity is a rare entity with few descriptions in the literature. Standard of care involves resection, either by Mohs micrographic surgery or wide local excision with careful evaluation of the surgical margins. Due to the invasive nature of this tumor, complete excision in sensitive areas such as the head and neck pose unique challenges.
We present a case of DFSP presenting from within the oral cavity, along with its management, in a 50-year-old male. In addition, we review the published literature. The patient neglected a 3-month history of a rapidly enlarging mass of the left cheek. A buccal mucosal biopsy revealed the diagnosis of DFSP. The patient underwent wide local excision of the tumor resulting in a full thickness defect which required reconstruction utilizing a radial forearm free flap. The final pathology report demonstrated positive margins that necessitated additional excision.
There are few reported cases of an intraoral presentation of dermatofibrosarcoma. Due to the sensitive location of the tumor, there are challenges associated with obtaining clear margins after resection and ultimately reconstruction.
This case and literature review highlight the rarity of an oral cavity presentation DFSP, and the associated surgical challenges.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的局部侵袭性软组织肿瘤，手术干预后复发率高。DFSP主要发生于躯干和四肢近端，据报道10%-15%发生于头颈部区域。口腔内出现的DFSP是一种罕见情况，文献中描述较少。治疗标准包括切除，可采用莫氏显微外科手术或广泛局部切除，并仔细评估手术切缘。由于该肿瘤具有侵袭性，在头颈部等敏感区域进行完整切除面临独特挑战。
我们报告一例50岁男性口腔内出现DFSP的病例及其治疗情况。此外，我们还回顾了已发表的文献。患者忽视了左颊部迅速增大肿物3个月的病史。颊黏膜活检确诊为DFSP。患者接受了肿瘤的广泛局部切除，导致全层缺损，需要采用桡侧前臂游离皮瓣进行重建。最终病理报告显示切缘阳性，需要再次切除。
口腔内出现皮肤纤维肉瘤的报道病例较少。由于肿瘤位置敏感，切除后获得清晰切缘并最终进行重建存在挑战。
本病例及文献回顾突出了口腔内出现DFSP的罕见性以及相关的手术挑战。
