Prostate Sarcoma: An Uncommon Diagnosis With Major Clinical Implications.

Prostate sarcomas are rare and aggressive malignancies. It commonly presents with obstructive urinary symptoms and a normal prostate-specific antigen (PSA), making early diagnosis difficult due to its clinical overlap with benign prostatic conditions. These tumors tend to present at an advanced stage due to unspecific symptoms and their aggressive progression. Radical surgery remains the gold-standard treatment, but its impact on the overall prognosis remains limited, particularly in patients over 50 years of age with metastatic or locally advanced disease. We present the case of a 71-year-old male who presented with refractory severe lower urinary tract symptoms (LUTS) despite a previous transurethral resection of the prostate (TURP) and dual therapy. Imaging revealed an enlarged prostate (300 g) and repeated TURP procedures failed to relieve symptoms or identify malignancy until the histopathology from a subsequent procedure revealed prostate leiomyosarcoma. Further imaging demonstrated local extension to the bladder and rectum. A multidisciplinary team proceeded with total pelvic exenteration, including resection of the prostate, bladder, and rectum, and creation of an ileal conduit and colostomy. Postoperative pathology was positive for a T4 leiomyosarcoma, infiltrating bladder and rectum, with preserved seminal vesicles. The patient initially recovered but later developed hypovolemic shock from arterial bleeding and required additional surgeries. Despite intensive care and surgical efforts, he died on postoperative day 28 due to disseminated intravascular coagulation. This case highlights the diagnostic challenges, aggressive progression, and scarcity of treatment options for this condition, emphasizing the importance of early diagnosis and multidisciplinary management.