Clinical and Pathological Features of Ewing Sacoma Family Tumors in Uro-oncology: A Single-Institute Experience

Abstract

Objectives: Genitourinary Ewing Sarcoma Family Tumors (ESFT) are rare and very aggressive tumors. We aimed todemonstrate the clinical features and prognosis of 6 genitourinary ESFT patients from a single institute from Turkey.Methods: A total of 6 cases of genitourinary ESFT (3 renal, 2 bladder and 1 adrenal) treated at our department werereviewed retrospectively and data considered with the literature.Results: Half of the patients in our cohort consisted of women and the median age of the cohort was 39 years at presentation.Median follow-up time was 46.3 (range; 3.6-103.2) months and median overall survival was 25 months. Halfof patients were diagnosed at metastatic stage. Four cases were undergone radical surgery, while the remaining caseswere performed diagnostic biopsy due to metastatic disease. Five cases received adjuvant or palliative chemotherapy,while one case (patient 5) rejected to receive chemotherapy.Conclusion: ESFT is rare among urologic malignancies, it is often overlooked for differential diagnosis preoperativelyand mortality is high despite multimodal treatment. We suggest that ESFT should be kept in mind as a rare differentialdiagnosis of all genitourinary tumors in advanced stage disease of young patients and large primary tumor especiallyif renal origin is involved.