Abstract
INTRODUCTION: Ewing sarcoma (ES) is a rare and aggressive neoplasm that primarily affects bones and soft tissues, with extraosseous manifestations extremely rare, making it difficult to diagnose and treat these localizations. CASE REPORT: A 32-year-old male experienced chronic pelvic pain and urinary symptoms due to a large prostatic ES (174 × 123 × 181 mm), resulting in acute renal failure. Despite normal PSA levels (1.5 ng/mL), imaging showed extensive local invasion. Histopathological examination revealed ES via CD99 positivity and molecular EWSR1 rearrangements. Palliative chemotherapy (VAC regimen) was given due to unresectability, but the patient died within eight months from disease progression. DISCUSSION: This case highlights the diagnostic challenges, essentially caused by non-specific symptoms and normal PSA levels, emphasizing the importance of immunohistochemical analysis for the confirmation. A review of the literature reveals that prostatic ES frequently presents in advanced stages, necessitating multimodal treatment. However, survival rates remain low despite the use of aggressive treatment. CONCLUSION: Prostate Ewing sarcoma is a formidable malignancy that must be detected early and treated aggressively. Increased clinician awareness and additional research into targeted therapies are critical for improving outcomes in this rare but aggressive disease.