眼窩内視神経全長に浸潤した年長児の網膜芽細胞腫の1例

Retinoblastoma metastatic to the intraorbital portion of optic nerve in an eight-year-old child

要約　背景：年長児の網膜芽細胞腫は，発見時には進行していることが多く，生存率が低いとされている。目的：8歳で発見された網膜芽細胞腫症例の報告。症例：8歳女児が3か月前からの白色瞳孔で受診した。視力は右1.5，左手動弁で，眼圧は右17mmHg，左33mmHgであった。右眼には異常がなく，左眼の水晶体後方に血管を伴う白色腫瘤と虹彩ルベオーシスがあった。CTで硝子体腔が石灰化のある腫瘤で満たされ，視神経が右眼の3倍に肥大していた。眼球を摘出し，網膜芽細胞腫の診断が確定した。視神経断端に腫瘍が陽性であった。化学療法と放射線照射を行い，初診から39か月後の現在まで，再発，全身転移，二次癌はない。結論：8歳で発見され，視神経転移がある網膜芽細胞腫でも，3年以上良好な経過をとることがある。

Abstract.　Background：Retinoblastoma detected in children at an advanced age may result in low survival rate due to extensive local or systemic involvement. Purpose：To report retinoblastoma diagnosed in an 8-year-old girl. Case and Findings：An 8-year-old girl presented with leukocoria since 3 months before. Visual acuity was 1.5 right and hand motion left. The right eye was free of abnormal findings. The left eye showed rubeosis iridis and vascularized white mass posterior to the lens. Computed tomography showed the vitreous to be filled with partially calcified mass. The left optic nerve had increased diameter throughout its intraorbital course. Enucleation confirmed the diagnosis of retinoblastoma with metastasis posterior to the cut end of optic nerve. She was treated by systemic chemotherapy and radiation. She has been doing well with no recurrence,systemic metastasis,or secondary malignancy for 39 months until present. Conclusion：The present case illustrates that retinoblastoma patient may survive for 3 years even after detection at 8 years of age.