Long-term disease control after upfront chemotherapy and surgery in a patient with primary prostate leiomyosarcoma.

INTRODUCTION: Prostate leiomyosarcoma is a rare, aggressive neoplasm. CASE PRESENTATION: A 52-year-old man presented with worsening frequent micturition and painful urination. Rectal examination revealed a significantly enlarged prostate. Magnetic resonance imaging showed a large prostate tumor with urinary bladder and bilateral seminal vesicle invasion. A prostate biopsy revealed diffuse proliferation of pleomorphic atypical cells. Immunohistochemistry confirmed the diagnosis of prostrate leiomyosarcoma. The patient received three cycles of the mesna, doxorubicin, ifosfamide, and dacarbazine regime (mesna 6000 mg/m2, doxorubicin 60 mg/m2, ifosfamide 7500 mg/m2, and dacarbazine 900 mg/m2) at 4-week intervals. The tumor shrank by 28% and exhibited necrotic changes. He underwent total pelvic exenteration with en bloc resection of the prostate, bladder, rectum, and anus. Pathological surgical margin was negative. The patient is alive with no disease at 5 years postoperatively. CONCLUSION: Neoadjuvant chemotherapy and surgical resection are essential to achieve a long-term survival of patients with localized prostate leiomyosarcoma.