Abstract

Ewing sarcoma/primitive neuroectodermal tumor (PNET) is the second most primary malignant bone tumor in children and adolescents between 10 and 25 years. The extraosseous sarcomas are rare and often cause diagnostic confusion for clinicians. The cell of origin is unclear, but exhibits rapid growth and recurrence, leading to a poor survival rate of 10% only. This imposes an increased risk of chronic health conditions, financial burden, and impaired quality of life. We report the uncommon presentation in age and site of the tumor in the peri-prostatic tissue of a 14-year-old boy and discuss our treatment approach. An exploratory observational case study was conducted. The ultrasound of an enlarged pelvis mass showed a large heterogeneously hyperechoic lesion with central necrosis and increased vascularity in the mass. The magnetic resonance imaging scan and histopathology of the transrectal ultrasound-guided biopsy specimen confirmed the prostate tumor. The transrectal biopsy proved to be a feasible and effective diagnostic tool for such an unclear pelvic mass. The extraskeletal manifestation of Ewing’s sarcoma imposes diagnostic confusion, but the case fulfilled the extraosseous Ewing sarcoma’s diagnostic characteristic. The Ewing’s sarcoma/PNET of the prostate being rare and posing a poor prognosis needs vigilant differential diagnosis and a multidisciplinary treatment approach for optimal and cost-effective treatments.

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