眼窩転移を伴う神経芽細胞腫の2症例

Two cases of metastatic neuroblastoma to the orbit

　眼球突出で発症した神経芽細胞腫の2症例を経験した。症例1は3歳4か月の男子で，乳児期スクリーニング検査では尿中のvanillylman—delic acid （VMA）とhomovanillic acid （HVA）は正常値であった。眼球突出は皮下出血を伴い急速に進行，精査にて両眼窩に転移した副腎原発の神経芽細胞腫と診断され，原発巣を摘出，化学療法で転移巣は縮小した。症例2は7か月の男子で，眼球突出と皮下出血で発症，精査にて眼窩に転移した副腎原発の神経芽細胞腫と診断され，化学療法を行ったが，3か月後に死亡した。皮下出血を伴い急速に進行する小児眼窩腫瘍では，神経芽細胞腫も考慮して検査を進める必要があると考える。

We report metastatic neuroblastoma to the orbit in 2 boys aged 3 years and 7 months respectively. The initial manifestation was rapidly developing exophthalmos with ecchymosis in both cases. The original lesion was stage IV-A neuroblastoma in the adrenal gland. In the first case, routine screen-ing for urine vanillylmandelic acid (VMA) and homovanillic acid (HVA) was negative at the ageof 6 months. Chemotherapy was effective and the exophthalmos regressed after surgical resection of the adrenal tumor. In the second case, exophthal-mos further progressed in spite of chemotherapy. He died 3 months later. The findings illustrate that metastatic orbital neuroblastoma may induce retrobulbar hemorrhage and progressive exophthal-mos. Neuroblastoma has to be ruled out first by urine VMA-HVA test in children with rapidly developing orbital tumor.