In this report, we describe the clinical, imaging, and pathologic find
ings in a case of diaphyseal telangiectatic osteosarcoma arising 23 ye
ars after successful treatment of bilateral retinoblastomas. To our kn
owledge, telangiectatic osteosarcoma occurring as a second tumor in th
is setting, outside the radiation treatment field, has not been previo
usly reported. The association of the RB-1 gene defect with retinoblas
tomas and other tumors is discussed. Also, the computed tomography and
magnetic resonance imaging findings in telangiectatic osteosarcoma ar
e described and discussed.