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Published on: May 1, 2015
[Retroperitonal fibrosis and lymphoma in a 15-year-old boy]
K Milcent1, S Franchi-Abella, S Larrar
1Service de pédiatrie générale, hôpital de Bicêtre, AP-HP, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre cedex, France. kmilcent@yahoo.fr
Summary
Retroperitoneal fibrosis (RF) is rare in children. This case highlights that unexplained RF in pediatric patients may indicate an underlying malignancy, such as anaplastic large cell lymphoma.
Area of Science:
- Pediatric Oncology
- Pediatric Nephrology
- Pediatric Rheumatology
Background:
- Retroperitoneal fibrosis (RF) is an uncommon condition characterized by inflammatory tissue in the retroperitoneum.
- While often idiopathic or associated with certain medications or conditions in adults, its presentation and etiology in children are less understood.
- Early diagnosis and appropriate management are crucial for favorable outcomes in pediatric patients.
Observation:
- A 15-year-old boy presented with persistent dorsal pain and fever.
- Elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) were noted.
- Imaging revealed a significant retroperitoneal mass, initially suspected to be RF.
Findings:
- A percutaneous lymph node biopsy confirmed anaplastic large cell lymphoma.
- This represents the first documented case of childhood RF as a manifestation of malignancy.
- The findings underscore the importance of a thorough oncological workup in pediatric RF.
Implications:
- Pediatric RF necessitates a comprehensive investigation to rule out underlying malignancies.
- This case broadens the differential diagnosis for retroperitoneal masses in children.
- Prompt identification of malignancy associated with RF can significantly alter treatment and prognosis.