The Ewing's Sarcoma Family of Tumors of Urinary Bladder: A Case Report and Review of the Literature

Abstract

Background: Only 15 cases of Ewing's Sarcoma (EWS) family of tumors of urinary bladder have been documented in the literature to date.Case Report: We presented here a 38 year-old female with primary urinary bladder EWS with no distant metastases. She had presented with macroscopic he-maturia and had undergone transurethral resection of the tumor within the following week. Microscopic ex-amination revealed a tumor diffusely infiltrating the lamina propria and muscularis propria under an in-tact urothelium, which was composed of small round blue cells with scant cytoplasm, monotonous round or oval nuclei, stippled chromatin and small nucleoli. Immunohistochemistry showed strong vimentin, syn-aptophysin and membranous CD99 expression by the tumor. Fluorescent in situ hybridization analysis displayed the EWSR1 rearrangement. Radical cystec-tomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy, extended lymph node dissec-tion, and ileal conduit were performed. As adjuvant chemotherapy, she received vincristine, doxorubicin, cyclophosphamide and mesna, alternating with cours-es of etoposide, iphosphamide and mesna. She is alive and well with no evidence of disease 14 months after the surgery.Conclusion: Surgery supported with chemotherapy should be considered as an option, especially in ad-vanced Ewing's sarcoma family of tumors of urinary bladder.Keywords: Ewing's Sarcoma, primitive neuroecto-dermal tumor, urinary bladder tumor