Leiomyosarcoma of lower urinary tract: Literature review

Leiomyosarcoma (LMS) of the lower urinary tract (LUT) is a rare and aggressive malignancy derived from smooth muscle cells. It most commonly arises in the bladder, is less frequent in the prostate, and exceptionally rare in the urethra. The disease typically affects adults around the sixth decade of life with a slight male predominance. Etiology remains unclear, although previous radiotherapy or exposure to specific chemotherapeutic agents has been implicated. Clinical presentation is often nonspecific and may resemble more prevalent urinary tract diseases, contributing to delayed or inaccurate diagnosis.

This narrative review summarizes current knowledge on LUT LMS based on available case reports and small case series. The literature was analyzed with focus on epidemiology, clinical features, diagnostic methods, and treatment strategies to highlight challenges in management and areas requiring further investigation.

Diagnosis of LUT LMS relies on histopathological assessment supported by immunohistochemistry, typically demonstrating positivity for smooth muscle markers such as smooth muscle actin, desmin, h-caldesmon, and vimentin. Surgery remains the cornerstone of treatment. Radical cystectomy is the most common approach for bladder tumors, while prostatic and urethral involvement often necessitates extensive surgical resection. The effectiveness of chemotherapy and radiotherapy is uncertain, and their use is tailored to individual tumor characteristics and patient condition. Regardless of location, prognosis is generally poor due to biological aggressiveness and frequent late presentation.

LUT LMS is an extremely rare malignancy with limited evidence available to guide management. Further research, including multicenter databases and prospective studies, is needed to establish prognostic factors and optimize treatment. Multidisciplinary collaboration is essential to ensure timely diagnosis, appropriate therapeutic planning, and improved patient outcomes.