Clinicopathological characteristics and favourable prognosis of paediatric cutaneous nodular fasciitis: a case series.

IF 3.7 4区 医学 Q1 DERMATOLOGY
Mutong Zhao, Xiaofeng Han, Li Wei, Juan Sun, Xu Li, Yuan Liang
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引用次数: 0

Abstract

Cutaneous nodular fasciitis (cNF) is a rare, benign myofibroblastic proliferation commonly misdiagnosed as a malignancy due to its rapid growth and histological features. We reviewed 15 paediatric patients with cNF, highlighting clinical, histological and molecular characteristics. The patients' median age was 6 years, and there was a predilection for cNF in the head and neck region. Histologically, cNF showed plump spindle cells and myxoid stromal changes. USP6 rearrangement, particularly MYH9-USP6 fusion, was present in the majority of patients. Spontaneous resolution was observed in nonsurgically managed cases. Accurate diagnosis by screening for USP6 rearrangement is essential to avoid overtreatment. This study underscores the benign nature of cNF and the importance of conservative management, especially in cosmetically sensitive areas.

小儿皮肤结节性筋膜炎的临床病理特征和良好预后:病例系列。
皮肤结节性筋膜炎(cNF)是一种罕见的良性肌成纤维细胞增生,由于其生长迅速和组织学特征,常被误诊为恶性肿瘤。本研究回顾了15例小儿结节性筋膜炎病例,强调了其临床、组织学和分子特征。中位年龄为6岁,好发于头颈部。组织学上,cNF表现为丰满的纺锤形细胞和肌样基质改变。大多数病例存在 USP6 基因重排,尤其是 MYH9-USP6 融合。在非手术治疗的病例中可观察到自然消退。通过 USP6 基因重排筛查进行准确诊断对于避免过度治疗至关重要。本研究强调了 cNF 的良性性质和保守治疗的重要性,尤其是在对美观敏感的部位。
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来源期刊
CiteScore
3.20
自引率
2.40%
发文量
389
审稿时长
3-8 weeks
期刊介绍: Clinical and Experimental Dermatology (CED) is a unique provider of relevant and educational material for practising clinicians and dermatological researchers. We support continuing professional development (CPD) of dermatology specialists to advance the understanding, management and treatment of skin disease in order to improve patient outcomes.
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