Metastatic retinoblastoma. Recent progress of interest to orthopaedic surgeons.

Retinoblastoma is the most common malignant intraocular neoplasm of childhood. Distant metastases to bone and bone marrow occur. The authors report on a retinoblastoma patient with metastases to the extraaxial skeleton. Implications for the orthopaedic surgeon and alternative diagnostic modalities are discussed. The case presented here exemplifies the potential role for the orthopaedic surgeon in making such a diagnosis in a child previously treated for retinoblastoma. Secondary nonocular malignant tumors should be considered in the differential diagnosis of patients with a history of retinoblastoma who have a painful extremity. Radiation treatment of the primary tumor increases the risk for nonocular secondary neoplasms. The structural alterations in the retinoblastoma gene in patients with osteosarcomas and the possible role of the retinoblastoma gene in the origin of osteosarcomas are also discussed.