Primary and secondary osteosarcoma of the face: a rare childhood malignancy.

BACKGROUND: Osteosarcoma of the head and neck, especially primary forms, remains a rare and highly malignant tumor. PATIENTS: This report describes two patients who developed an osteosarcoma of the face more than ten years after treatment for bilateral retinoblastoma. We also report a third patient who presented with a primary osteosarcoma of the right superior maxilla which is one of the rarest tumors encountered in childhood oncology. CONCLUSIONS: The mainstay of therapy is surgical resection with negative margins. Careful, long-term follow-up of survivors of hereditary retinoblastoma is essential, especially for those given radiation therapy