Leiomyosarcoma of the maxilla as second malignancy in retinoblastoma

Patients with hereditary retinoblastoma are at increased risk of second primary tumor, the commonest tumor being osteosarcoma. Leiomyosarcoma developing as second primary neoplasm in retinoblastoma patients is unusual and most have occurred in the field of previous radiotherapy. Although with aggressive therapy better survival can be achieved, the overall prognosis of patients developing these second neoplasms is poor. In this report we present a case of leiomyosarcoma of the maxilla as a second neoplasm in a patient with bilateral retinoblastoma which has developed outside the radiation field.