小儿原发性皮纤维肉瘤的手术治疗:关于两个具有挑战性的病例报告

IF 1.4 Q3 PEDIATRICS Global Pediatric Health Pub Date : 2024-09-27 eCollection Date: 2024-01-01 DOI:10.1177/2333794X241286916
Yacine Zouirech, Abir Manni, Badr Rouijel, Ahmed El Baoudi, Hajar El Agouri, Nawfal Fejjal
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引用次数: 0

摘要

原发性皮肤纤维肉瘤(DFSP)是一种极为罕见的儿童皮肤肿瘤,其特点是局部侵袭性强、生长缓慢、复发率高且转移潜力低。由于其生长缓慢且经常被误诊,其在儿童中的发病率往往被低估。由于症状不具特异性,诊断 DFSP 具有挑战性。虽然大多数病例表现为躯干或四肢近端结节性病变,但有些病变,如萎缩性斑块或硬化性结节斑块,可与血管畸形相仿,使临床医生感到困惑。组织学和免疫组化研究对明确诊断至关重要。首选的治疗方法是彻底手术切除,并保留较宽的切缘,以降低复发风险。我们报告了两例小儿躯干 DFSP 病例,其中一例模仿血管畸形。两例病例均成功切除了 4 厘米的边缘,分别在 6 个月和 2 年的随访后未发现复发。连续监测将持续至少 5 年。
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Surgical Management of Pediatric Dermatofibrosarcoma Protuberans: About Two Challenging Case Reports.

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.

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来源期刊
Global Pediatric Health
Global Pediatric Health Nursing-Pediatrics
CiteScore
2.20
自引率
0.00%
发文量
105
审稿时长
12 weeks
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