Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Bladder in a Young Male: A Rare Case Report and Literature Review

Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, commonly located in the kidney, uterus, and soft tissues. Their occurrence in the urinary bladder is extremely uncommon, with fewer than 40 cases reported in the literature to date. Case Presentation: We describe a 20-year-old male who presented with hematuria and was diagnosed with a malignant PEComa of the bladder. The patient underwent partial cystectomy, pelvic lymph node dissection, and uretero-ureterostomy. Histopathological evaluation revealed epithelioid cells with immunohistochemical positivity for HMB-45, Melan-A, SMA, and cathepsin K, confirming the diagnosis. Postoperative management included 25 sessions of adjuvant radiotherapy. No evidence of recurrence was observed during one year of follow-up. Conclusion: This case highlights the importance of early recognition and aggressive surgical management in patients with malignant bladder PEComa. We also provide a comprehensive review of the literature to contextualize our findings and inform clinicians on optimal diagnostic, therapeutic, and follow-up strategies.