Maes P, Brichard B, Vermylen C, Cornu G, Ninane J
Department of Pediatric Hematology and Oncology, Cliniques Universitaires Saint-Luc, Catholic University of Brussels, Belgium.
Med Pediatr Oncol. 1998 Mar;30(3):170-4. doi: 10.1002/(sici)1096-911x(199803)30:3<170::aid-mpo8>3.0.co;2-e.
Osteosarcoma of the head and neck, especially primary forms, remains a rare and highly malignant tumor.
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.
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.