2世代にわたり治療した網膜芽細胞腫の8家系

Eight pedigrees of retinoblastoma treated over two generations

1967～2005年に筆者のひとりが治療した8家系16名の親子の網膜芽細胞腫患者につき，初診時年齢，病気の進行度，治療法などを比較検討した。39年間にわたる本疾患に関する変遷としては，初診時年齢は親の世代では中央値が6か月で，子の世代では1か月であった。進行眼の病期は親ではReese-Ellsworth分類でⅤ期であり，子の世代ではⅢ期であった。初回治療としての眼球摘出は，親では50％，子では6％であった。眼球保存療法の成功率は親では57％，子では71％であり，子の世代の観察期間が短いが治療法の進歩が示唆された。子の世代での早期受診が確認されたが，親が患者で本疾患に関する知識が十分にあるはずなのに，生後すぐに受診せず平均で生後1か月である現状は問題であり，生後1週間以内に眼底検査を受けるように十分に指導を徹底すべきであるとともに，何らかの方法で親に受診を勧告する方法を検討する必要があると思われた。

To report 16 patients（a child and the father or the mother）of 8 families with retinoblastoma who were treated by the first author from 1967 to 2005. Their age and stage at the first visit,therapies and outcomes were compared between the generation of parents and children to recognize progress of medical and social aspects during 39 years. The median age at the first visit of parents was 6months old and that of their children was one month old. The median stage of the more advanced eye at the first visit was Reese-Ellsworth Group Ⅴ in the parents and that of the children was Group Ⅲ. Enucleation of the eye ball was selected as the initial treatment in 50％ of parents versus 6％ of children. The success rate of eye-preservation therapy was 57％ in parents and 71％ in children. The age of the first visit of children must be less than one week,because their parents had bilateral retinoblastoma and they were supposed to well understand the autosomal dominant heredity of their retinoblastoma. Some social system must be developed to promote earlier fundus examination of their babies. And more effective methods for eye-preservation therapy of advanced stages are mandatory to increase their success rate.