Malignant fibrous histiocytoma of maxilla following radiotherapy for bilateral retinoblastoma

A 20-year-old man developed a malignant fibrous histiocytoma in the right maxilla 19 years after irradiation for bilateral retinoblastoma. The incidence of second tumours in patients who survived bilateral retinoblastoma treated with radiation was 8.5 per cent. Malignant fibrous histiocytomas which arise in a site of prior radiation are fatal. The present case is presumed to have the autosomal dominant retinoblastoma gene, not associated with deletion of the q14 band of chromosome 13. The patient succumbed to the second tumour. (author)