A case of osteogenic sarcoma of left nasal cavity following treatment for familial retinoblastoma.

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A case of osteogenic sarcoma of the left nasal cavity following treatment for familial bilateral retinoblastoma is reported.A 7-year-old boy with left nasal obstruction and bad odor from the nose was referred to our hospital for diagnosis and treatment.When he was 10 month old, he was found to have bilateral retinoblastoma, and Linac 5200 rads irradiation, cryosurgery and bilateral enucleation were performed. An elder brother, a cousin and two paternal uncles had retinoblastoma.The secondary tumor occupied the left nasal cavity and ethmoid sinus, and there was calcification around the middle turbinate. The pathologic examination revealed osteogenic sarcoma. Denker's operation, tumor resection with dura resection and dura plasty under lateral rhinotomy, repeated reduction operation, 60Co 5000 rads irradiation and chemotherapy were performed, but the tumor always recurred at the primary site.The incidence of retinoblastoma is increasing. Despite the high survival rate of patients with retinoblastoma, most secondary malignant tumors are fatal. We emphasize that patients treated for retinoblastoma must be observed carefully to detect secondary tumors at an early stage.
耳鼻咽喉科臨床学会の論文