Bone and soft-tissue sarcoma risk in long-term survivors of hereditary retinoblastoma treated with radiation Journal Article


Authors: Kleinerman, R. A.; Schonfeld, S. J.; Sigel, B. S.; Wong-Siegel, J. R.; Gilbert, E. S.; Abramson, D. H.; Seddon, J. M.; Tucker, M. A.; Morton, L. M.
Article Title: Bone and soft-tissue sarcoma risk in long-term survivors of hereditary retinoblastoma treated with radiation
Abstract: PURPOSE Survivors of hereditary retinoblastoma have excellent survival but substantially increased risks of subsequent bone and soft-tissue sarcomas, particularly after radiotherapy. Comprehensive investigation of sarcoma risk patterns would inform clinical surveillance for survivors. PATIENTS AND METHODS In a cohort of 952 irradiated survivors of hereditary retinoblastoma who were originally diagnosed during 1914 to 2006, we quantified sarcoma risk with standardized incidence ratios (SIRs) and cumulative incidence analyses. We conducted analyses separately for bone and soft-tissue sarcomas occurring in the head and neck (in/near the radiotherapy field) versus body and extremities (out of field). RESULTS Of 105 bone and 124 soft-tissue sarcomas, more than one half occurred in the head and neck (bone, 53.3%; soft tissue, 51.6%), one quarter in the body and extremities (bone, 29.5%; soft tissue, 25.0%), and approximately one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). We noted substantially higher risks compared with the general population for head and neck versus body and extremity tumors for both bone (SIR, 2,213; 95% CI, 1,671 to 2,873 v SIR, 169; 95% CI, 115 to 239) and soft-tissue sarcomas (SIR, 542; 95% CI, 418 to 692 v SIR, 45.7; 95% CI, 31.1 to 64.9). Head and neck bone and soft-tissue sarcomas were diagnosed beginning in early childhood and continued well into adulthood, reaching a 60-year cumulative incidence of 6.8% (95% CI, 5.0% to 8.7%) and 9.3% (95% CI, 7.0% to 11.7%), respectively. In contrast, body and extremity bone sarcoma incidence flattened after adolescence (3.5%; 95% CI, 2.3% to 4.8%), whereas body and extremity soft-tissue sarcoma incidence was rare until age 30, when incidence rose steeply (60-year cumulative incidence, 6.6%; 95% CI, 4.1% to 9.2%), particularly for females (9.4%; 95% CI, 5.1% to 13.8%). CONCLUSION Strikingly elevated bone and soft-tissue sarcoma risks differ by age, location, and sex, highlighting important contributions of both radiotherapy and genetic susceptibility. These data provide guidance for the development of a risk-based screening protocol that focuses on the highest sarcoma risks by age, location, and sex. Copyright © 2019 American Society of Clinical Oncology. All rights reserved.
Journal Title: Journal of Clinical Oncology
Volume: 37
Issue: 35
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 2019-12-10
Start Page: 3436
End Page: 3445
Language: English
DOI: 10.1200/jco.19.01096
PUBMED: 31622129
PROVIDER: scopus
DOI/URL:
Notes: Article -- Source: Scopus
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  1. David H Abramson
    272 Abramson