Molecular profile & clinical outcome in 121 cases - experience from a tertiary referral centre in South India

Authors : Kiruthiga K G , Anne Jennifer Prabhu, Rekha Pai, Leni G Mathew, Selvamani Backianathan

DOI : 10.18231/j.ijpo.2023.033

Volume : 10

Issue : 2

Year : 2023

Page No : 163-169

Background: Ewing sarcoma is the second most common sarcoma involving the bones in children and adolescents. Published data on the clinical features, morphology, translocation and follow-up of patients with Ewing sarcoma from India, is sparse. Objectives of this study were to analyse the clinico-pathologic features of Ewing sarcoma and compare with translocation status, BCoR immunohistochemistry, treatment and survival.
Materials and Methods: 406 cases of Ewing sarcoma were diagnosed in the Department of Pathology, Christian Medical College, Vellore during the period 2008-2015. 135 patients underwent molecular testing for common translocations by RT-PCR and were included in this study.
Results: Mean age of patients at diagnosis was 22 years. Most common location of tumour was appendicular skeleton (31.4%) and 19% had solid organ involvement. Distant metastasis was present in 21.5% patients. Translocation was seen in 63 (46.7%) cases, EWS-FLI1 type I (87%), EWS-FLI1 type II(11%) and one patient had EWS-ERG translocation. Seven out of 30 patients were classified as “Sarcoma with BCOR genetic alteration”. Patients who underwent 6 cycles of chemotherapy had a better mean survival. Mean follow-up was 13.72 months and 3 year event free survival of patients was 93.8%.
Conclusions: Percentage of viable tumour <10>  

Keywords: BCoR, CD99, Ewing sarcoma, FLI1, Translocation.


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