Related Experiment Video
Updated: May 5, 2026

A Patient-Derived Xenograft Model for Venous Malformation
Published on: June 15, 2020
Pediatric eosinophilic granulomatosis with polyangiitis presenting as a mediastinal mass in a 6-year-old boy
Sagar Bishowkarma1, Suraj Shrestha1, Roshan Aryal1
1Mahrajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.
Insights
Eosinophilic granulomatosis with polyangiitis (EGPA) is rare in children. This case highlights EGPA presenting as mediastinal lymphadenopathy, a unique presentation requiring careful diagnosis in pediatric patients.
Area of Science:
- Pediatric Rheumatology
- Rare Diseases
- Immunology
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small vessel vasculitis.
- Pediatric EGPA is uncommon, posing diagnostic challenges.
- Mediastinal mass presentation in pediatric EGPA is exceptionally rare.
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.
Related Concept Videos
Differentiation of Common Myeloid Progenitor Cells
Skin Cancer
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Disorders of Leukocytes
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
Primary Lymphoid Organs
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
Pulmonary Tuberculosis III
The first classification is based on the development of the disease, and it includes the following categories:
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

