Pediatric embryonal rhabdomyosarcoma of the prostate: Lessons from a rare case

Embryonal rhabdomyosarcoma (eRMS) of the prostate is rare and typically presents in boys under 10 years. We report a 15-year-old boy with DICER1 syndrome incidentally found to have a prostatic mass incidentally found during ER evaluation. Magnetic resonance imaging (MRI) revealed marrow metastases occult on computed tomography (CT), and fluorodeoxyglucose-positron emission tomography (PET)/CT confirmed widespread disease. Biopsy demonstrated eRMS without FOXO1 rearrangement. This case highlights the importance of MRI and PET/CT for staging and management in pediatric prostate rhabdomyosarcoma.