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

Journal of Clinical Oncology Dec 13, 2019

Kleinerman RA, Schonfeld SJ, Sigel BS, et al. - Researchers measured sarcoma risk, with standardized incidence ratios and cumulative incidence analyses, in a cohort including 952 irradiated survivors of hereditary retinoblastoma who were basically diagnosed from 1914 to 2006. Separate analyses were performed for bone and soft-tissue sarcomas happening in the head and neck (in/near the radiotherapy field) vs body and extremities (out of field). There were 105 bone and 124 soft-tissue sarcomas, with the occurrence of more than one half noted 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 about one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). For both bone and soft-tissue sarcomas, substantially higher risks than the general population were reported for head and neck vs body and extremity tumors. For head and neck bone and soft-tissue sarcomas, a 60-year cumulative incidence of 6.8% and 9.3%, respectively, was estimated. Flattening of body and extremity bone sarcoma incidence was noted following adolescence, whereas a rare incidence of body and extremity soft-tissue sarcoma was noted until age 30, when incidence increased steeply, especially for females. In this study, findings highlighted variations in the strikingly raised bone and soft-tissue sarcoma risks by age, location, and gender, implying significant contributions of both radiotherapy and genetic susceptibility.
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