Kengne Ulrich Igor Mbessoh, Thiam Jaafar Ibn Abou Talib, Fall Amacoumba, Balde Salif, Ndiaye Mamadou, Mekontso Joel Gabin Konlack, Sarr Gorgui, Zoure Etienne Tossou, Sow Mamadou, Ka Sidy
Department of Surgical Oncology, Dalal Jamm National Hospital Center, Dakar, Senegal.
Department of Surgery and Specialties, Faculty of Medicine, Pharmacy and Odontostomatology, Cheikh Anta Diop University, Dakar, Senegal.
Case Rep Oncol Med. 2025 May 8;2025:9991548. doi: 10.1155/crom/9991548. eCollection 2025.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma originating from fibroblasts in the dermal connective tissue, comprising approximately 1% of all soft tissue sarcomas. While most cases involve the trunk and extremities, only 10%-15% occur in the cephalic region, representing less than 1% of all head and neck neoplasms. DFSP is notable for its high propensity for local recurrence following surgical excision and its low metastatic potential. We report a case of recurrent DFSP of the forehead extending to the anterior wall of the left frontal sinus, without brain involvement, in a 33-year-old male with a history of three prior wide local excisions. A multidisciplinary cancer team recommended systemic imatinib therapy. This case highlights the challenges of managing DFSP in an uncommon location, underscoring the importance of a multidisciplinary approach in addressing recurrent and complex presentations.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的软组织肉瘤,起源于真皮结缔组织中的成纤维细胞,约占所有软组织肉瘤的1%。虽然大多数病例发生在躯干和四肢,但只有10%-15%发生在头部区域,占所有头颈部肿瘤的比例不到1%。DFSP的显著特点是手术切除后局部复发倾向高,转移潜能低。我们报告一例33岁男性复发性前额DFSP,病变延伸至左额窦前壁,未累及脑部,该患者既往有三次广泛局部切除病史。一个多学科癌症治疗团队建议采用伊马替尼全身治疗。该病例凸显了在不常见部位管理DFSP的挑战,强调了多学科方法在处理复发性和复杂病例中的重要性。