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 q 14 band of chromosome 13. The patient succumbed to the second tumour.
Chondrosarcoma of the nasal septum is an uncommon lesion. Review of the English literature reveals only 14 cases. This paper reports an additional case observed in a Japanese series of seven cases. The clinical features are reviewed, and a most effective treatment with minimum side effects is organized by an interdisciplinary group of specialists in surgery, radiotherapy and regional chemotherapy.
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