Abstract

Introduction: Ewing Family Tumour (EFT) is a group of rare malignant and aggressive tumour, with a considerably improved prognosis. However, there is lack of study on the outcome of children with EFT in Malaysia. Objectives: The study aimed to evaluate the Overall Survival (OS) rate, Event Free Survival (EFS) rate and identify the prognostic factors that determined the EFT outcome at Hospital Universiti Sains Malaysia (USM). Methodology: A retrospective record review of children aged 0-18 years with EFT was done. Patients were identified from the registration data in the Oncology Unit and Record Office of Hospital USM. For patients with untraceable information or deceased, a letter was sent to State Registry to obtain the outcome of the patient. The association between demographics and patients’ clinical factors was determined using the Cox regression. Survival curves were estimated by the Kaplan-Meier method and were compared using the Log-rank test. Results: There were 51 patients identified but 29 of them were eligible for the study. The mean duration of follow-up was 21 months. The OS rate at 1, 2, 3 and 5 years were 62.1%, 44.8%, 30.2% and 21.6% respectively. The EFS rate at 1, 2, 3 and 5 years were 41.9%, 26.7%, 17.8% and 0% respectively. Multivariate Cox regression analysis showed that the presence of surgical intervention (p = 0.030) and major complications (p = 0.045) were the significant prognostic factors to the survival of EFT. Conclusion: The survival rate of EFT among our patients was comparable to other developing countries, with surgical intervention and the presence of major complications as independent prognostic factors.

Highlights

  • Ewing Family Tumour (EFT) is a group of rare malignant and aggressive tumour, with a considerably improved prognosis

  • There were 51 children aged 0-18 years who were diagnosed with Ewing Family Tumour (EFT) from 1st January 2002 to 31st December 2012

  • Two of the confirmed EFT cases were excluded because one patient was managed in the other hospital and another one was diagnosed outside the study period

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Summary

Introduction

Ewing Family Tumour (EFT) is a group of rare malignant and aggressive tumour, with a considerably improved prognosis. Objectives: The study aimed to evaluate the Overall Survival (OS) rate, Event Free Survival (EFS) rate and identify the prognostic factors that determined the EFT outcome at Hospital Universiti Sains Malaysia (USM). Conclusion: The survival rate of EFT among our patients was comparable to other developing countries, with surgical intervention and the presence of major complications as independent prognostic factors. The incidence of EFT varies among the different racial groups, ages and is much rarely seen in Asian It has a peak incidence at the age of 15 years and is more common in males compared to females, with the ratio of 1.3 to 1.5: 1. Malaysian Journal of Paediatrics and Child Health (MJPCH) | (December 2021) | Page 70 of 78 between the date of diagnosis and the earliest occurrence of the following events: induction failure, relapse, death from any cause, last contact and development of a second malignancy [5]. Hospital Universiti Sains Malaysia (USM) has provided a multidisciplinary approach to musculoskeletal and bony oncology since 1990’s

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