Survival and prognostic factors of childhood Ewing sarcoma - Experience from a cancer center in South India.

Abstract

Background: Childhood Ewing sarcoma (ES) - both osseous and extraosseous - has good outcomes with aggressive treatment that includes chemotherapy, surgery, and radiation therapy (RT). In this paper, we report our experience on the survival and prognostic factors of ES.

Materials and methods: Seventy-four newly diagnosed patients with ES, less than 14 years of age, registered in Pediatric Oncology Department of our center, over a period of 5 years, from July 1, 2010 to June 30, 2015, were included.

Results: Mean age at presentation was 8.93 years. Primary osseous sites were the extremity (n = 30), pelvis (n = 10), chest wall (n = 9), spine (n = 7), and skull (n = 5), and the extraosseous primary site was involved in 17.6% (n = 13). Metastatic disease was present in 17 patients (23%). Also, 52 patients received three-weekly vincristine/doxorubicin/cyclophosphamide (VDC)/ifosfamide/etoposide (IE) and 21 patients received only VDC. There was no treatment-related mortality. Local control modalities were surgery alone (n = 12), definitive RT (n = 33), surgery and RT (n = 20), palliative RT (n = 4), or none (n = 4). At a median duration of follow-up of 64 months, the 5-year event-free survival (EFS) for the entire cohort was 50.4% (60.6% for localized and 17.6% for metastatic) and the 5-year overall survival (OS) was 54.6% (64.2% for localized and 23.5% for metastatic). Among the prognostic factors analyzed (age, gender, lactate dehydrogenase [LDH] levels, site, and metastases), presence of metastases was the only factor significantly associated with poorer EFS and OS.

Conclusion: Localized ES has a favorable outcome even in resource-limited settings, and treatment intensification by interval-compressed chemotherapy may improve the outcomes further. Presence of metastasis at presentation confers a poor outcome.