ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical characteristics, diagnosis and treatment of urinary neurofibrosarcoma.</p><p><b>METHODS</b>Clinical data of 1 case of urinary neurofibrosarcoma was analyzed retrospectively and related literature reviewed. The 61-year old male patient had undergone TURP for benign prostate hyperplasia ten months before.</p><p><b>RESULTS</b>Total urethral cavernostomy, total cystectomy and ileal cystostomy were performed on the patient and pathological diagnosis pointed to urinary neurofibrosarcoma. Another operation was done for metastatic rectal mass, which revealed abdominal metastasis. With progressing consumptive constitution, the patient died 40 days after the second operation.</p><p><b>CONCLUSION</b>It is difficult to make a definite diagnosis in urinary neurofibrosarcoma, and the effective treatment for this highly malignant disease needs to be further studied.</p>