Pediatric eosinophilic granulomatosis with polyangiitis presenting as a mediastinal mass in a 6-year-old boy

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by asthma, eosinophilia, and small vessel necrotizing granulomatosis with annual incidence of 0.5-4.2 cases per million. EGPA is uncommon in childhood and only a handful of pediatric cases have been reported in the literature making it difficult to diagnose. EGPA in the pediatric population presenting with mediastinal mass is extremely rare. Here, we present a rare case of pediatric EGPA with unique presentation i.e. mediastinal lymphadenopathy in a 6-year-old boy who presented with recurrent episodes of cough and shortness of breath. On further evaluation, patient was found to have mediastinal lymphadenopathy and the diagnosis of pediatric EGPA was confirmed by histopathology of the mass. High dose steroid was started at 1mg/kg/day and then gradually tapered to maintenance dose and weaned off after remission. Diagnosis of EGPA in pediatric population is challenging and requires careful differentiation under mimicking conditions. Though uncommon, EGPA can cause mediastinal lymphadenopathy and should be considered a differential diagnosis.

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Publication Details

Journal
Radiology Case Reports
Published
2025-03-08
DOI
https://doi.org/10.1016/j.radcr.2025.01.060
Primary Topic
Eosinophilic Disorders and Syndromes
Type
article
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article

Pediatric eosinophilic granulomatosis with polyangiitis presenting as a mediastinal mass in a 6-year-old boy

Roshan Aryal, Suraj Shrestha, Pradeep Raj Regmi, Sagar Bishowkarma et al.
Radiology Case Reports
Eosinophilic Disorders and Syndromes
article

Pediatric eosinophilic granulomatosis with polyangiitis presenting as a mediastinal mass in a 6-year-old boy

Roshan Aryal, Suraj Shrestha, Pradeep Raj Regmi, Sagar Bishowkarma, Isha Amatya, Anu Radha Twayana, Bikash Kumar Mishra, Kundan Kumar Yadav
article en

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by asthma, eosinophilia, and small vessel necrotizing granulomatosis with annual incidence of 0.5-4.2 cases per million. EGPA is uncommon in childhood and only a handful of pediatric cases have been reported in the literature making it difficult to diagnose. EGPA in the pediatric population presenting with mediastinal mass is extremely rare. Here, we present a rare case of pediatric EGPA with unique presentation i.e. mediastinal lymphadenopathy in a 6-year-old boy who presented with recurrent episodes of cough and shortness of breath. On further evaluation, patient was found to have mediastinal lymphadenopathy and the diagnosis of pediatric EGPA was confirmed by histopathology of the mass. High dose steroid was started at 1mg/kg/day and then gradually tapered to maintenance dose and weaned off after remission. Diagnosis of EGPA in pediatric population is challenging and requires careful differentiation under mimicking conditions. Though uncommon, EGPA can cause mediastinal lymphadenopathy and should be considered a differential diagnosis.

Radiology Case ReportsVol. 20(5)
Nepal Medical College Teaching Hospital (NP), Tribhuvan University Teaching Hospital (NP), Nepal Health Research Council (NP), Kathmandu University (NP)
Openalex Percentile: Top 94%
Eosinophilic Disorders and Syndromes
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Pediatric eosinophilic granulomatosis with polyangiitis presenting as a mediastinal mass in a 6-year-old boy — Roshan Aryal, Suraj Shrestha, et al. · Radiology Case Reports (2025) | TGRS Research Map | TGRS