Undifferentiated pleomorphic prostate sarcoma: a rare clinical case and systematic literature review

Abstract

Undifferentiated pleomorphic prostate sarcoma is an extremely rare neoplasm. In the available literature, less than 50 clinical cases were described. Usually, the clinical manifestation of the disease is nonspecific and is primarily associated with bladder outlet obstruction. Additionally, most patients have normal levels of prostate-specific antigen, while ultrasound and MRI can visualize irregularly shaped lesions, often extending beyond the prostate gland. Diagnosis requires exclusion of other signs of specific differentiation, apart from fibroblastic and myofibroblastic. There is no established treatment plan for patients with pleomorphic prostate sarcoma due to extreme rarity of the disease. For localized forms of the disease, radical laparoscopic prostatectomy is often used, but more aggressive treatment options are also being considered, including total pelvic exenteration, chemotherapy and radiation therapy. However, in most cases metastasis is revealed after primary treatment. Regional metastases are often localized in the rectum, bladder, in the area of previous surgery, and regional lymph nodes, while distant metastases are found in the lungs, liver, bones and distant lymph nodes, thereby indicating aggressive progression and poor prognosis of the disease.In this article, we present our own clinical observation of a patient with confirmed undifferentiated pleomorphic prostate sarcoma.