Clinical characteristics of nodular fasciitis of the ear in children

The rate of early misdiagnosis in patients with nodular fasciitis of the ear is high. To provide a basis for clinical diagnosis and treatment, we aimed to summarise the clinical manifestations, imaging results, pathological findings, treatment strategies, and postoperative follow-up results for three cases of paediatric nodular fasciitis (two girls, one boy) treated in the Department of Otorhinolaryngology, Head and Neck Surgery, at Beijing Children's Hospital of Capital Medical University from 2018 to 2020. The average age at diagnosis was 24 months. Lesions occurred in the left ear in two cases and right ear in one case. All patients had a history of biopsy before surgery. Rapid growth was observed following biopsy in two patients, and anti-inflammatory treatment was ineffective in all three cases. Fluorescence in situ hybridisation analysis of ubiquitin-specific peptidase 6 (USP6) was performed in two of the three cases, with positive results. The lesions exhibited hypo-intensity or iso-intensity on T1-weighted magnetic resonance imaging (MRI) and heterogeneous hyper-intensity on T2-weighted MRI. "Fascial tail" signs were observed on imaging in all cases. Surgical resection was performed in all cases. Intact ear appearance was observed at follow-up, and there were no cases of recurrence.Conclusion: Combining clinical features with imaging findings may improve the accuracy of preoperative diagnosis in patients with nodular fasciitis. In addition to pathological findings, genetic testing for USP6 may aid in diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence. What is Known: • Paediatric NF around the ear is rare and is easily misdiagnosed as other inflammatory masses that have a higher incidence in children. • Most previous reports of NF were case reports. What is New: • Combining clinical and imaging findings with genetic testing for USP6 rearrangement may improve the accuracy of preoperative diagnosis in patients with NF. Nonetheless, the final diagnosis should be based on comprehensive assessment. • The present paper is significant in that it represents the only report of three cases of ear NF in children with a complete medical history and prognosis.