Erdheim-Chester disease mimicking Wilms tumor in a child: a diagnostic challenge.

Abstract excerpt

BACKGROUND: Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis driven by activating mutations of the MAPK signaling pathway, most commonly BRAF V600E. Pediatric cases are exceedingly rare and often present with atypical, multisystem manifestations, resulting in significant diagnostic delay. Renal and retroperitoneal involvement may mimic primary renal malignancies, particularly Wilms tumor....

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