Abstract

Simple SummaryThe treatment of osteosarcoma and Ewing sarcoma, the two major types of malignant bone tumors in children, has progressed considerably during the last decades, with more patients becoming long-term survivors. This improvement has resulted in an increasing number of patients with long-term adverse health consequences from the life-saving treatment. The aim of this study was to provide a detailed, comprehensive overview of somatic diseases that require hospitalization in long-term survivors of osteosarcoma and Ewing sarcoma. This study contributes new insights into the risk of somatic late effects in survivors of osteosarcoma and Ewing sarcoma which are urgently requested by pediatric oncologists, researchers, and by survivors and their families. The study provides an essential basis for the development of preventive intervention strategies and for optimal patient counseling and follow-up care, which all contribute to improving the health and quality of life in survivors.Survivors of malignant bone tumors in childhood are at risk of long-term adverse health effects. We comprehensively reviewed cases of somatic diseases that required a hospital contact in survivors of osteosarcoma and Ewing sarcoma. In a population-based cohort study, 620 five-year survivors of osteosarcoma (n = 440) or Ewing sarcoma (n = 180), diagnosed before the age of 20 years in Denmark, Finland, Iceland, and Sweden during 1943–2008, were followed in the national hospital registers. Overall rates of hospital contacts for any somatic disease and for 12 main diagnostic groups and 120 specific disease categories were compared with those in a matched comparison cohort (n = 3049) randomly selected from the national population registers. The rate of hospital contact for any somatic disease was 80% higher in survivors of malignant bone tumors than in comparisons and remained elevated up to 30 years after diagnosis. The rate of hospital contacts was higher after Ewing sarcoma (rate ratio (RR) 2.24; 95% confidence interval (CI) 1.76–2.85) than after osteosarcoma (RR 1.67; 95% CI 1.41–1.98). Elevated rates were observed for 11 main diagnostic groups, including infections, second malignant neoplasms, and diseases of the skin, bones, and circulatory, digestive, endocrine, and urinary systems. Survivors of malignant bone tumors in childhood are at increased risk of somatic diseases many years after diagnosis. This comprehensive study contributes new insight into the risk of late effects in survivors of osteosarcoma and Ewing sarcoma, which is an essential basis for optimal patient counseling and follow-up care.

Highlights

  • IntroductionOsteosarcoma and Ewing sarcoma are the commonest primary malignant bone tumors in children and adolescents, accounting for approximately 6% of all childhood cancers [1,2]

  • Osteosarcoma and Ewing sarcoma are the commonest primary malignant bone tumors in children and adolescents, accounting for approximately 6% of all childhood cancers [1,2].The commonest site for both tumors is the extremities, but more Ewing sarcomas than osteosarcomas occur in the axial skeleton [1,2]

  • A total of 537 hospital contacts were observed among the 620 survivors of a childhood malignant bone tumor during follow-up, and survivors had an 80% higher rate of hospital contacts for any disease than comparisons (RR 1.80; 95% confidence interval (CI) 1.56–2.08; Table 2)

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Summary

Introduction

Osteosarcoma and Ewing sarcoma are the commonest primary malignant bone tumors in children and adolescents, accounting for approximately 6% of all childhood cancers [1,2]. The commonest site for both tumors is the extremities, but more Ewing sarcomas than osteosarcomas occur in the axial skeleton [1,2]. Chemotherapy and surgery are the primary therapy for osteosarcoma, as the tumor is not responsive to radiation at conventional doses [2]. Ewing sarcoma is controlled locally with surgery, radiation therapy, or a combination of the two [1]. Most bone tumors in the extremities were managed by amputation, advances in high-dose chemotherapy and in surgical techniques have made limb salvage the leading surgical procedure [2]

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