ARTICLE

Patients with retinoblastoma have excellent survival; up to 97 % will survive 5 years ( 1). However, survivors of hereditary retinoblastoma have substantially increased risks of developing subsequent pri-mary malignancies, including soft tissue sarcomas ( 2 – 8). The predis-position to soft tissue sarcomas in retinoblastoma survivors has been attributed to a germline mutation in the RB1 gene ( 9), which encodes the cell cycle regulatory retinoblastoma protein (pRb). Alterations in the retinoblastoma pathway have also been identified in a variety of soft tissue sarcomas in patients without a history of retinoblastoma ( 10 – 14). In a previous study of a cohort of patients with hereditary reti-noblastoma ( 3), we reported a strong radiation dose – response relationship for soft tissue sarcomas, primarily in the head and neck region, such that patients treated for retinoblastoma with 60 Gy or more had 11 times the risk of patients treated with les