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