Abstract
Prostate leiomyosarcoma is an extremely rare and very aggressive neoplasm that represents less than 0.1% of primary malignant tumors of the prostate. We present a patient with primary leiomyosarcoma of prostate and examine the cases reported in the literature to discuss the clinical, diagnostic and therapeutic aspects of this rare tumor. Median survival was estimated at 17 months (95% CI 20.7–43.7 months) and the actuarial survival rates at 1, 3 and 5 years were 68%, 34% and 26%, respectively. The only predictors of long-term survival were negative surgical margins and the absence of metastatic disease at presentation. A multidisciplinary approach is necessary for the proper management of this terrible entity.
 Surgery with or without chemotherapy seems to be the main therapeutic method for operable leimyosarcomas, but in general there is no consensus on the best therapeutic approach. Most cases are diagnosed at an advanced stage of the disease. A global multicenter trial is needed to find therapies that would improve the prognosis.
Highlights
Leiomyosarcoma of the prostate is an extremely rare malignant tumor that represents less than 0.1% of primary malignant tumors of the prostate [1]
Its diagnosis is generally made at a late stage, because of the clinical and radiological non-specificity. It is often bulky beyond the prostate gland and invading neighboring organs
Prostate leiomyosarcoma has been reported in children and adults ranging from 2.5 years to 80 years of age [6]
Summary
Leiomyosarcoma of the prostate is an extremely rare malignant tumor that represents less than 0.1% of primary malignant tumors of the prostate [1] It is the most common primary sarcoma of the prostate in adults and comprises 38% to 52% of primary prostate sarcomas [2]. Its diagnosis is generally made at a late stage, because of the clinical and radiological non-specificity. It is often bulky beyond the prostate gland and invading neighboring organs. Figure 2: anatomopathological examination showing a partially necrotic fusocellular tumor proliferation with positive, strong, and diffuse immunostaining of antidesmine AC tumor cells in favor of prostatic leiomyosarcoma
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