Arfa Wajdi, Bouhajja Leila, Ghammem Mohamed, Amri Mohamed, Farah Faten, Jlalia Zied, Jenzri Mourad
Mohammed Kassab National Institute of Orthopaedics Pediatric orthopedic department Kssar Said La Mannouba Tunisia.
University of Tunis El Manar Faculty of Medicine of Tunis Tunis Tunisia.
Clin Case Rep. 2025 Jan 31;13(2):e9497. doi: 10.1002/ccr3.9497. eCollection 2025 Feb.
Dermato fibrosarcoma protuberans presents challenges in diagnosis and treatment, especially in children. Awareness of its aggressive local recurrence and its potential for multifocal presentation is crucial. Wide surgical resection with adequate margins remains the basis of management, in association with regular follow-up of affected patients.
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma, particularly uncommon in children. We report the case of a 13-year-old boy who initially presented with a firm mass on the left foot, later diagnosed as DFSP following histological and immunohistochemical analysis. The tumor was surgically excised with a wide margin, and a skin graft was used for coverage. Despite an initial favorable outcome, a new DFSP lesion developed at the proximal left thigh 1 year later, requiring further wide surgical excision and coverage with a tensor fascia lata flap. Both sites remained free of recurrence at one-year follow-up. This case underscores the importance of long-term vigilance in managing DFSP due to the potential risk of recurrence and multifocal involvement.
隆突性皮肤纤维肉瘤在诊断和治疗方面存在挑战,尤其是在儿童中。认识到其侵袭性局部复发及多灶性表现的可能性至关重要。广泛手术切除并保证足够切缘仍是治疗的基础,同时要对受影响患者进行定期随访。
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的、局部侵袭性皮肤肉瘤,在儿童中尤为少见。我们报告一例13岁男孩,最初表现为左足有一坚实肿块,经组织学和免疫组化分析后诊断为DFSP。肿瘤行广泛手术切除并带足够切缘,用皮肤移植覆盖创面。尽管最初预后良好,但1年后左大腿近端出现新的DFSP病灶,需再次广泛手术切除并用阔筋膜张肌皮瓣覆盖创面。在1年随访时,两个部位均无复发。该病例强调了由于存在复发和多灶性累及的潜在风险,在管理DFSP时长期保持警惕的重要性。