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I.はじめに
本邦における小児の頭蓋内胚細胞腫瘍(Germ Cell Tumors,GCTs)は,小児脳腫瘍の約10%を占め,欧米に比較して数倍多いと言われている.その中でも胎児蛋白であるα—fetoprotein(AFP)を産生するYolk Sac Tumorは最も悪性の性格を示し,従来は放射線治療が第一選択として行われてきたが,予後不良で殆どが1年以内に再発している.
しかし,近年小児の睾丸・卵巣領域のnonseminoma—tous GCTsに対してCisplatin(CDDP),Vinblastine(VBL),Bleomycin(BLM)を含むPVB療法8),さらにこれら三剤にActinomycin D(AMD),Cyclophospha—mide(CPM)を加えたVAB−6療法2,6)(Table 1)の有効性が報告され,頭蓋内GCTsに対しても試みられている1,4,7,12).
Several clinical trials have demonstrated that cispla-tin-based chemotherapy for primary intracranial germ-cell tumors is effective as a neoadjuvant chemotherapy.
In this report, we describe a 6-year-old boy, Down syndrome and Hirschsprung's disease with intracranial pure yolk sac tumor treated by combined chemother-apy with cisplatin, vinblastine, bleomycin and cy-clophosphamide (modified VAB-6 regimen). He had been admitted to our hospital because of intractable vomiting, and left facial nerve palsy since lmonth be-fore. An MRI revealed an enlarged mass, 4cm in dia-meter, in the left cerebello-pontine angle with uniforml enhancement by Gd-DTPA, and bilateral ventricular dilatation. He was found to have increased serum alpha-fetoprotein level (AFP 11, 786ng/ml), but not hu-man chorionic gonadotropin β-subunit. After a partial resection of the tumor, diagnosed as pure yolk sac tumor, and ventriculo-peritoneal shunt, three courses of combined chemotherapy with cisplatin, bleomycin, vinblastine and cyclophosphamide (modified VAB-6 therapy) were carried out. The serum AFP level re-turned to normal, and the tumor mass entirely dis-appeared (a complete response) on MRI after the second course of chemotherapy. However, cisplatin-induced vomiting and mild neutropenia and renal tubu-lar injury developed after the third course of che-motherapy. Irrespective of administration of recom-binant human G-CSF and broad spectrum antibiotics, he suffered from pneumonia and died of septic shock and multiple organ failure. Autopsy showed microsco-pic residual tumors.
The combination chemotherapy with cisplatin, bleomycin, vinblastine and cyclophosphamide is effective for initial treatment of childhood intracranial yolk sac tumor. It is necessary, however, to reevaluate the cis-platin dosage and treatment schedule in order to reduce such side effects as bone marrow suppression and renal damage.
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