Renal Ewing's sarcoma: A single institute experience of seven patients.

Abstract

e22538 Background: Renal Ewing’s sarcoma constitutes a rare subset of extraskeletal ewings with aggressive clinical course and dismal outcomes. The purpose of our study was to review our experience in the management of patients with Renal Ewing’s Sarcoma. Methods: We retrospectively analysed patients with Renal Ewing’s Sarcoma who were registered in Sarcoma Medical Oncology Clinic at AIIMS, New Delhi between September 2016 and January 2019. Results: A total of eight patients were registered in the study period, one of which was diagnosed with wilms tumor post nephrectomy and was excluded from our study. Of seven patients included in analysis, five (71.4%) were males. The commonest symptoms were flank pain (57.1%) and hematuria (57.1%). The median age was 28 years (range, 18 to 50 years). The mean Hemoglobin was 10.4 g/dl (range, 6-14 g/dl). The median tumor size was 13 cm (range, 5-25 cm). Four patients (57.1%) were non-metastatic while three (42.9%) had metastases in bone, lung and liver, and peritoneum respectively. Diagnosis was established by an upfront nephrectomy in four patients (57.1%) and biopsy in three patients (42.9%). Translocation for EWSR1 was done in 5 patients of which four (80%) were positive and one patient (20%) were negative. The test was not carried out in two patients due to inadequate tissue sampling. Three patients received neoadjuvant chemotherapy with VAC and the rest (four) received it after surgery. Ifosfamide-etoposide (IE) was added to VAC regimen in first two patients, in one being added post nephrectomy. This patient developed Ifosfamide-induced renal failure resulting in death. Radiotherapy was given in two patients, one pre-operative owing to large inoperable mass, and post operative in the other due to positive margins. At last follow up, three patients (42.9%) are alive and are on chemotherapy after radical nephrectomy. Four patients (57.1%) have died, three due to disease (all metastatic) and one due to chemotoxicity. The median survival was 15 months. Conclusions: Unlike extremity, renal ewings sarcoma more commonly presents in higher age and with advanced disease. Translocation studies are a prerequisite in a suspected case of Renal Ewings Sarcoma, especially due to possibility of discrepancies in pre and post-operative diagnoses. Our institutional protocol does not recommend the use of Ifosfamide in the subset of patients with single kidney, post nephrectomy. Caution must be exercised before instituting the drug and should be given only pre-operatively in absence of renal dysfunction.