Zouirech Yacine, Manni Abir, Rouijel Badr, El Baoudi Ahmed, El Agouri Hajar, Fejjal Nawfal
Pediatric Plastic surgery Unit, Children's Hospital of Rabat, Morocco.
Faculty of Medicine and Pharmacy, Mohamed V University of Rabat, Morocco.
Glob Pediatr Health. 2024 Sep 27;11:2333794X241286916. doi: 10.1177/2333794X241286916. eCollection 2024.
Dermatofibrosarcoma protuberans (DFSP) is an extremely rare cutaneous tumor in children, marked by local aggressiveness, slow growth, high recurrence rate, and low metastatic potential. Its prevalence is often underestimated in children due to its slow growth and frequent misdiagnosis. Diagnosing DFSP can be challenging due to nonspecific symptoms. While most cases present as nodular lesions on the trunk or proximal extremities, some lesions, such as atrophic plaques or sclerotic nodular plaques, can mimic vascular malformations and confuse clinicians. Histologic and immunohistochemical studies are essential for definitive diagnosis. The treatment of choice is complete surgical resection with wide margins to reduce the risk of recurrence. We report two pediatric DFSP cases on the trunk, including one mimicking vascular malformations. Both cases had successful 4 cm margin resections, with no recurrences observed after 6 months and 2 years of follow-up, respectively. Continuous surveillance will be maintained for at least 5 years.
隆突性皮肤纤维肉瘤（DFSP）是一种极其罕见的儿童皮肤肿瘤，其特点是局部侵袭性、生长缓慢、复发率高和转移潜能低。由于其生长缓慢且经常误诊，其在儿童中的患病率常常被低估。由于症状不具特异性，诊断DFSP可能具有挑战性。虽然大多数病例表现为躯干或近端肢体上的结节性病变，但一些病变，如萎缩性斑块或硬化性结节性斑块，可类似血管畸形并使临床医生感到困惑。组织学和免疫组化研究对于明确诊断至关重要。首选的治疗方法是进行广泛切缘的完整手术切除，以降低复发风险。我们报告了两例发生在躯干的儿童DFSP病例，其中一例类似血管畸形。两例均成功进行了4厘米切缘的切除，分别在随访6个月和2年后未观察到复发。将持续监测至少5年。
