Osteosarcoma as a second malignant disease in a case of bilateral retinoblastoma

Patients with hereditary retinoblastoma, whether treated by radiation or not, are at an increased risk of subsequently developing sarcomas as a second malignancy. As the understanding of the underlying genetics of osteosarcoma and retinoblastoma has improved, a clear association between the two entities has been noted. We present a case of a young patient with previous history of bilateral retinoblastoma who was treated by surgery and chemoradiation for his primary disease and subsequent recurrences. Thirteen years after his initial diagnosis, he developed osteosarcoma of the mandible as the second primary lesion and was treated with surgery and chemotherapy. The intent of the case report is to bring forth the current thinking of genetic association between retinoblastoma and osteosarcoma and to discuss aggressive treatment protocol for the latter, in the form of surgery and chemotherapy.