Kleinerman RA, Schonfeld SJ, Sigel BS, et al. - Given the excellent survival reported in survivors of hereditary retinoblastoma but with substantially increased risks of subsequent bone and soft-tissue sarcomas, particularly after radiotherapy, researchers sought to determine sarcoma risk patterns for survivors as it may assist in their clinical surveillance. In this cohort study of 952 irradiated survivors of hereditary retinoblastoma, 105 bone and 124 soft-tissue sarcomas were identified; of these 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 nearly one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). Diagnosis of head and neck bone and soft-tissue sarcomas began in early childhood and continued well into adulthood, reaching a 60-year cumulative incidence of 6.8% and 9.3%, respectively. In contrast, they observed flattening in body and extremity bone sarcoma incidence after adolescence (3.5%; 95% CI, 2.3% to 4.8%), whereas diagnosis of body and extremity soft-tissue sarcoma was made rarely until age 30, when its 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%). Findings thereby indicate differences in strikingly elevated bone and soft-tissue sarcoma risks in correlation with age, location, and gender. This shows significant contributions of both radiotherapy and genetic susceptibility. These data may assist in developing a risk-based screening protocol focusing on the highest sarcoma risks by age, location, and gender.