{"id":158,"date":"2012-04-19T01:59:46","date_gmt":"2012-04-18T16:59:46","guid":{"rendered":"\/sawamura\/wp\/?p=158"},"modified":"2023-10-11T14:48:20","modified_gmt":"2023-10-11T05:48:20","slug":"atrt","status":"publish","type":"post","link":"https:\/\/plaza.umin.ac.jp\/sawamura\/pediattumor\/atrt\/","title":{"rendered":"AT\/RT(atypical teratoid\/rhabdoid tumor)\u3000\u30e9\u30d6\u30c9\u30a4\u30c9\u816b\u760d"},"content":{"rendered":"<div class=\"setumei\">\n<h5><span style=\"color: #000080;\"><strong>\u975e\u5b9a\u578b\u5947\u5f62\u816b\u69d8\uff0f\u30e9\u30d6\u30c9\u30a4\u30c9\u816b\u760d<br \/>\nembryonal tumor WHO grade 4<\/strong><\/span><\/h5>\n<ul>\n<li>AT\/RT\u306f\u65e5\u672c\u8a9e\u306e\u9069\u5207\u306a\u8a33\u304c\u3042\u308a\u307e\u305b\u3093\u304b\u3089\uff0c\u305d\u306e\u307e\u307e<span style=\"color: #000080;\"><strong><span style=\"color: #ff0000;\">AT\/RT\uff08\u30a8\u30fc\u30c6\u30a3\u30fc\u30fb\u30a2\u30fc\u30eb\u30c6\u30a3\u30fc\uff09<\/span>\u3068\u547c\u3070\u308c\u307e\u3059<\/strong><\/span><\/li>\n<li>\u80ce\u5150\u671f\u306e\u7d30\u80de\u304b\u3089\u767a\u751f\u3059\u308b\u3068\u8003\u3048\u3089\u308c\u3066\u3044\u307e\u3059<\/li>\n<li><strong><span style=\"color: #ff0000;\">\u4e73\u5150\u3084\u5e7c\u5150<\/span><\/strong>\u306b\u3067\u304d\u308b\u816b\u760d\u3067<span style=\"color: #ff0000;\"><strong>3\u6b73\u672a\u6e80\u306b\u591a\u3044<\/strong><\/span>\u3067\u3059\uff0c\u8d64\u3061\u3083\u3093\u306e\u8133\u816b\u760d\u3068\u3082\u8a00\u3048\u307e\u3059<\/li>\n<li><strong><span style=\"color: #000080;\">\u767a\u75c7\u5e74\u9f62\u4e2d\u592e\u5024\u306f1\u6b73\u534a\u304f\u3089\u3044\u3067\uff0c80-90\uff05\u306f3\u6b73\u672a\u6e80<\/span><\/strong>\u3067\u3059<\/li>\n<li><span style=\"color: #333333;\">1\u6b73\u672a\u6e80\u306e\u60aa\u6027\u8133\u816b\u760d\u306e50%\u307b\u3069<\/span>\u306b\u306a\u308a\u307e\u3059<\/li>\n<li><em>SMARCB1<\/em>\u907a\u4f1d\u5b50\u7523\u7269<span style=\"color: #ff0000;\"><strong>\u00a0INI-1 (<\/strong><\/span><span style=\"color: #333333;\"><span style=\"color: #ff0000;\"><strong>\u30a4\u30cb\u30ef\u30f3)\u00a0<\/strong><\/span>\u306b\u7570\u5e38\u304c\u3042\u308a\u307e\u3059<\/span><\/li>\n<li>\u5c0f\u8133\u306b\u591a\u3044\u306e\u3067\u3059\u304c\uff0c\u8133\u306e\u3069\u306e\u90e8\u5206\u306b\u3082\u767a\u751f\u3059\u308b\u816b\u760d\u3067\u3059<\/li>\n<li>\u75c7\u72b6\u3084\u753b\u50cf\u306a\u3069\u304c\u4f3c\u3066\u3044\u308b\u305f\u3081\u306b\u9ac4\u82bd\u816b\u3084PNET\u3068\u8aa4\u8a3a\u3055\u308c\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u982d\u84cb\u5185\u5727\u4ea2\u9032\u75c7\u72b6\u3068\u3044\u3046<span style=\"color: #000080;\"><strong>\u982d\u75db\u3084\u5614\u5410\uff0c\u610f\u8b58\u969c\u5bb3\uff08\u53cd\u5fdc\u304c\u306b\u3076\u3044\uff09<\/strong><\/span>\u3067\u767a\u75c7\u3059\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li>\u767a\u75c7\u3057\u305f\u6642\u306b\u306f\u3059\u3067\u306b<span style=\"color: #000080;\"><strong>\u5de8\u5927\u306a\u816b\u760d<\/strong><\/span>\u306b\u306a\u3063\u3066\u3044\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li>\u9ac4\u6db2\u306b\u306e\u3063\u3066\u8133\u306e\u4ed6\u306e\u90e8\u5206\u3084<span style=\"color: #000080;\"><strong>\u810a\u9ac4\u306b\u8ee2\u79fb\uff08\u9ac4\u6db2\u64ad\u7a2e\uff09\u3057\u307e\u3059<\/strong><\/span><\/li>\n<li>\u8a3a\u65ad\u306fMRI\u3067\u3057\u307e\u3059\u304c\uff0c\u6700\u521d\u304b\u3089\u5fc5\u305a\u810a\u9ac4\u306e\u65b9\u3082\u8abf\u3079\u306a\u3051\u308c\u3070\u306a\u308a\u307e\u305b\u3093<\/li>\n<li>\uff13\u304b\u3089\uff14\u5272\u3067<span style=\"color: #000080;\"><strong>\u8a3a\u65ad\u6642\u306b\u3059\u3067\u306b\u64ad\u7a2e<\/strong><\/span>\u304c\u751f\u3058\u3066\u3044\u307e\u3059<\/li>\n<li>\u6b8b\u5ff5\u306a\u304c\u3089<span style=\"color: #000080;\"><strong>\u60aa\u6027\u5ea6\u304c\u9ad8\u304f\u3066\u6cbb\u7642\u3082\u96e3\u3057\u3044<\/strong><\/span>\u3082\u306e\u3067\u3059<\/li>\n<li>\u534a\u6570\u4ee5\u4e0a\u306e\u5b50\u4f9b\u305f\u3061\u304c<span style=\"color: #000080;\"><strong>1\u5e74\u4ee5\u5185\u3067\u4ea1\u304f\u306a\u308a\u307e\u3059<\/strong><\/span><\/li>\n<li><span style=\"color: #ff0000;\"><strong>2021\u5e74\u306e\u6642\u70b9\u3067\uff0c\u4e16\u754c\u7684\u30b3\u30bb\u30f3\u30b5\u30b9\u304c\u3042\u308b\u6cbb\u7642\u65b9\u6cd5\uff08\u6a19\u6e96\u6cbb\u7642\uff09\u306f\u3042\u308a\u307e\u305b\u3093<\/strong><\/span><\/li>\n<li>\u307e\u305a\u624b\u8853\u3067\u3067\u304d\u308b\u3060\u3051\u3068\u308a\u307e\u3059\u304c\uff0c<span style=\"color: #000080;\"><strong>\u3068\u308a\u5207\u308c\u305f\u6642\u306b\u52a9\u304b\u308b\u53ef\u80fd\u6027\u304c\u9ad8\u304f\u306a\u308a\u307e\u3059<\/strong><\/span><\/li>\n<li>\u624b\u8853\u3067\u53d6\u308a\u5207\u308c\u305f\u3068\u601d\u3063\u3066\u3082<span style=\"color: #000080;\"><strong>\u3042\u3063\u3068\u3044\u3046\u9593\u306b\u518d\u767a<\/strong><\/span>\uff08\u518d\u71c3\uff09\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059\u304b\u3089\uff0c\u5316\u5b66\u7642\u6cd5\u3084\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u6025\u304e\u307e\u3059<\/li>\n<li>\u5316\u5b66\u7642\u6cd5\u3068\u3044\u3063\u3066\u3082<span style=\"color: #000080;\"><strong>\u5927\u91cf\u306e\u5236\u304c\u3093\u5264<\/strong><\/span><span style=\"color: #000000;\">\u3068<span style=\"color: #333333;\">\u9ac4\u6ce8\uff08<\/span><\/span><span style=\"caret-color: #000080; color: #333333;\">\u9ac4\u6db2\u8154\u5185\u3078\u85ac\u3092\u5165\u308c\u308b\uff09<\/span><span style=\"color: #ff0000;\"><span style=\"color: #000000;\">\u3092\u4f7f\u3046<\/span><\/span>\u3082\u306e\u3067\u3059<\/li>\n<li>\u5316\u5b66\u7642\u6cd5\u3068\u3057\u3066\u306f\u3001<span style=\"color: #ff0000;\"><strong>IRS-III\u3068\u304bACNS0333\u306e\u30ec\u30b8\u30e1\u30f3<\/strong><\/span>\u3068\u3044\u3046\u65b9\u6cd5\u306b\u6e96\u3058\u305f\u3082\u306e\u304c\u671f\u5f85\u3055\u308c\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong>\u653e\u5c04\u7dda\u6cbb\u7642\u304c\u3067\u304d\u305f\u4f8b\u3067\u9577\u671f\u751f\u5b58\u304c\u5831\u544a<\/strong><\/span>\u3055\u308c\u3066\u3044\u307e\u3059\u304c\uff0c\u4f4e\u5e74\u9f62\u306a\u306e\u3067\u653e\u5c04\u7dda\u969c\u5bb3\u3082\u5927\u304d\u304f\u3067\u307e\u3059<\/li>\n<li>\uff13\u6b73\u672a\u6e80\u306e\u5b50\u4f9b\u305f\u3061\u306b\u306f<span style=\"color: #000080;\"><strong>\u3067\u304d\u308c\u3070\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u3057\u306a\u3044\u3067\u5316\u5b66\u7642\u6cd5\u3060\u3051\u3067\u6cbb\u7642\u3059\u308b<\/strong><\/span>\u3068\u3044\u3046\u9078\u629e\u80a2\u304c\u3068\u3089\u308c\u307e\u3057\u305f<\/li>\n<li>\u3067\u3082\u305d\u308c\u3067\u306f\u52a9\u304b\u3089\u306a\u3044\u3053\u3068\u3082\u591a\u3044\u306e\u3067\uff0c<span style=\"color: #000080;\"><strong>\u8a3a\u65ad\u6642\u306b\u64ad\u7a2e\u304c\u306a\u3044\u4f8b\u3067\u306f\uff13\u6b73\u672a\u6e80\u306e\u4f4e\u5e74\u9f62\u3082\u542b\u3081\u3066\u5c40\u6240\u7167\u5c0454\u30b0\u30ec\u30a4<\/strong><\/span>\u3092\u9078\u3073\u307e\u3059<\/li>\n<li>\u5c40\u6240\u7167\u5c04\u3068\u3044\u3048\u3069\u3082\u3001\u5e74\u9f62\u3068\u816b\u760d\u306e\u90e8\u4f4d\u306b\u3088\u3063\u3066\u306f\u4f7f\u3048\u306a\u3044\u3053\u3068\u3082\u3042\u308a\u307e\u3059<\/li>\n<li>\u4e73\u5e7c\u5150\u3092\u542b\u3081\u3066<span style=\"color: #000080;\"><strong>\u9577\u671f\u751f\u5b58\u4f8b\u3067\u306fup-front radiation therapy\uff08\u653e\u5c04\u7dda\u6cbb\u7642\u304b\u3089\u306f\u3058\u3081\u308b\uff09\u304c\u306a\u3055\u308c\u3066\u3044\u305f<\/strong><\/span>\u3068\u3044\u3046\u5831\u544a\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u4e73\u5e7c\u5150\u306e\u8133\u5ba4\u5185\u306b\u3042\u308aINI1\u86cb\u767d\u7570\u5e38\u304c\u3042\u308b\u3082\u306e\u3067\u306f\uff0c\u826f\u6027\u306e\u7d4c\u904e\u3092\u305f\u3069\u308b cribriform neuroepithelial tumor CRINET \u3092\u8003\u3048\u308b\u5fc5\u8981\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u4e00\u65b9\u3067\uff0c\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u884c\u3046\u3068\u77e5\u80fd\u4e88\u5f8c\uff08\u8a8d\u77e5\u969c\u5bb3\uff09\u304c\u304b\u306a\u308a\u3064\u3089\u3044\u72b6\u614b\u3068\u306a\u308b\u306e\u3067\uff0c\u6709\u52b9\u3068\u89e3\u3063\u3066\u3044\u3066\u3082\u4f7f\u7528\u3057\u306a\u3044\u3068\u3044\u3046\u610f\u898b\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u3053\u306e\u3088\u3046\u306b\uff0c\u6cbb\u7642\u6cd5\u306e\u9078\u629e\u306b\u306f\u77db\u76fe\u304c\u3044\u3063\u3071\u3044\u3067\u3059<\/li>\n<li>\u3042\u307e\u308a\u306b\u3082\u72b6\u614b\u304c\u53b3\u3057\u3044\u5834\u5408\u3067\u306f\uff0c\u4f55\u3082\u6cbb\u7642\u3057\u306a\u3044\u3067\u7de9\u548c\u30b1\u30a2\u306e\u307f\u3092\u3059\u308b\u3068\u3044\u3046\u3053\u3068\u3082\u9078\u629e\u80a2\u3067\u3042\u308b\u8133\u816b\u760d\u3067\u3059<\/li>\n<\/ul>\n<h4>AT\/RT\u306eMRI\u753b\u50cf<\/h4>\n<p><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/ATRTMRI.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-full wp-image-3017\" title=\"ATRTMRI\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/ATRTMRI.jpg\" alt=\"\" width=\"460\" height=\"243\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/ATRTMRI.jpg 460w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/ATRTMRI-300x158.jpg 300w\" sizes=\"auto, (max-width: 460px) 100vw, 460px\" \/><\/a><\/p>\n<p>AT\/RT\u306eMRI\u50cf\u306f\u3055\u307e\u3056\u307e\u3067\u3059\u3002\u4e73\u5e7c\u5150\u306egrade IV\u306e\u60aa\u6027\u816b\u760d\u3067\u3059\u304b\u3089\uff0c\u5897\u5927\u901f\u5ea6\u304c\u6975\u3081\u3066\u901f\u304f\uff0c\u5de8\u5927\u3067\uff0c\u816b\u760d\u5468\u8fba\u8133\u6d6e\u816b\u304c\u5f37\u304f\uff0c\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u3067\u5f37\u304f\u5897\u5f37\u3055\u308c\u308b\u3068\u3044\u3046\u306e\u304c\u4e00\u822c\u7684\u3067\u3059\u3002\u786c\u819c\u3092\u4fb5\u3057\u982d\u84cb\u9aa8\u7834\u58ca\u3092\u4f34\u3063\u3066\u982d\u84cb\u304b\u3089\u5916\u306b\u81a8\u9686\u3057\u3066\u304f\u308b\u306e\u3082\u3042\u308a\u307e\u3059\u3002\u591a\u304f\u306f\u816b\u760d\u5185\u90e8\u306b\u58ca\u6b7b\u304c\u898b\u3089\u308c\u58ca\u6b7b\u6027\u306e\u3046\u80de\u304c\u5f62\u6210\u3055\u308c\u307e\u3059\u3002CT\/MRI\u3067\u3001\u816b\u760d\u8840\u6d41\u304c\u8c4a\u5bcc\u3067\u7d30\u80de\u5bc6\u5ea6\u304c\u9ad8\u3044\u3068\u3044\u3046\u6240\u898b\u306f\u91cd\u8981\u306a\u70b9\u3067\u3059\u3002<br \/>\n\u3067\u3082\u3053\u306e\u4f8b\uff08\u4e0a\u306e\u753b\u50cf\uff09\u306e\u69d8\u306b\uff0cmass effect\u3082\u8efd\u5ea6\u3067\u816b\u760d\u5468\u8fba\u6d6e\u816b\u3082\u76ee\u7acb\u305f\u306a\u3044\u3082\u306e\u3082\u73cd\u3057\u304f\u306f\u3042\u308a\u307e\u305b\u3093\u3057\uff0c<span style=\"color: #000080;\"><strong>\u4ed6\u306e\u80ce\u5150\u6027\u8133\u816b\u760d<\/strong><strong><span style=\"color: #000080;\">\u3068\u306f<\/span>\u753b\u50cf\u8a3a\u65ad\u3067\u9451\u5225\u3059\u308b\u3053\u3068\u306f\u3067\u304d\u307e\u305b\u3093<\/strong><\/span>\u3002<\/p>\n<h4>\u6cbb\u7642\u306e\u65b9\u5411\u6027<\/h4>\n<ul>\n<li>\u60aa\u6027\u5ea6\u306e\u3068\u3066\u3082\u9ad8\u3044\u816b\u760d\u306a\u306e\u3067\uff0c\u52a9\u304b\u308b\u305f\u3081\u306b\u306f\u4f55\u3067\u3082\u3057\u306a\u3051\u308c\u3070\u306a\u3089\u306a\u3044\u3068\u3044\u3046\u8003\u3048\u306b\u3082\u7acb\u3061\u307e\u3059<\/li>\n<li>\u6cbb\u7642\u306f\u4e00\u6982\u306b\u306f\u8a9e\u308c\u307e\u305b\u3093\uff0c\u767a\u751f\u3057\u305f\u5e74\u9f62\uff0c\u8133\u306e\u90e8\u4f4d\uff0c\u64ad\u7a2e\u8ee2\u79fb\u304c\u3042\u308b\u304b\u3069\u3046\u304b\u3067\u5927\u304d\u304f\u5909\u308f\u3063\u3066\u3057\u307e\u3046\u304b\u3089\u3067\u3059<\/li>\n<li>\u5916\u79d1\u7684\u7acb\u5834\u304b\u3089\u306f\uff0c\u521d\u671f\u8a3a\u65ad\u5f8c\u306b\u53ef\u80fd\u3067\u3042\u308c\u3070\u958b\u982d\u624b\u8853\u3067\u5168\u6458\u51fa\u3057\u307e\u3059<\/li>\n<li>\u653e\u5c04\u7dda\u5316\u5b66\u7642\u6cd5\u5f8c\u306b\u6b8b\u5b58\u816b\u760d\u304c\u3042\u308c\u3070\u305d\u308c\u3092\u5168\u6458\u51fa\u3059\u308b\u3053\u3068\u3092\u7a4d\u6975\u7684\u306b\u8003\u616e\u3057\u307e\u3059 (second look surgery)<\/li>\n<li>\u64ad\u7a2e\u304c\u3042\u308b\u5834\u5408\u306b\u306f\uff0c\u6cbb\u7642\u3092\u3057\u306a\u3044\u3067\u7de9\u548c\u30b1\u30a2\u3092\u3060\u3051\u3092\u3059\u308b\u3068\u3044\u3046\u3082\u306e\u6709\u529b\u306a\u9078\u629e\u80a2\u3067\u3059<\/li>\n<\/ul>\n<h4>AT\/RT\u306e\u5316\u5b66\u7642\u6cd5<\/h4>\n<ul>\n<li>\u304b\u3064\u3066\u306e\uff0cPediatric Oncology Group (POG)9233\/4 \u3068 Children\u2019s Cancer Group (CCG) 9921\u3067\u306e2\u5e74EFS\u306f6.4%\u3068\u4f4e\u3044\u3082\u306e\u3067\u3057\u305f<\/li>\n<li>2020\u5e74\u6642\u70b9\u3067\uff0cBoston Protocol, EU-RHAB, ACNS0333\u306a\u3069\u306e\u30d7\u30ed\u30c8\u30b3\u30fc\u30eb\u304c\u7528\u3044\u3089\u308c\u3066\u3044\u307e\u3059<\/li>\n<li>MTX\u306e\u9ac4\u6ce8\u3092\u3057\u306a\u3044\uff0c\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3092\u7528\u3044\u308b\uff0c\u8133\u810a\u9ac4\u7167\u5c04\u3067\u306f\u306a\u304f\u53ef\u80fd\u3067\u3042\u308c\u3070\u5c40\u6240\u7167\u5c04\u3092\u7528\u3044\u308b\u3068\u3044\u3046\u65b9\u5411\u6027\u306b\u3042\u308a\u307e\u3059<\/li>\n<li>\u5e74\u9f62\uff0c\u816b\u760d\u306e\u5e83\u304c\u308a\uff0c\u624b\u8853\u5f8c\u306e\u816b\u760d\u30b5\u30a4\u30ba\u306a\u3069\u306b\u3088\u3063\u3066\u4fee\u98fe modification\u3055\u308c\u307e\u3059<\/li>\n<\/ul>\n<\/div>\n<h4><span style=\"color: #ff0000;\">ACNS0333 protocol<\/span>\u306e\u6982\u8981<\/h4>\n<p>Reddy AT: Efficacy of High-Dose Chemotherapy and\u00a0Three-Dimensional Conformal Radiation for\u00a0Atypical Teratoid\/Rhabdoid Tumor: A Report From\u00a0the Children\u2019s Oncology Group Trial ACNS0333. J Clin Oncol 2020<\/p>\n<ul>\n<li>\u8133\u810a\u9ac4\u7167\u5c04\u3092\u3057\u306a\u3044\u3067<span style=\"color: #000080;\"><strong>\u5c40\u6240\u7167\u5c04 local radiation\u3092\u5fdc\u7528<\/strong><\/span>\u3057\u3088\u3046\u3068\u3059\u308b\u8a66\u307f\u3067\u3059<\/li>\n<li>\u624b\u8853\u5f8c\u306b2\u30b3\u30fc\u30b9\u306e\u5316\u5b66\u7642\u6cd5 (VCR, MTX, VP-16, CY, CDDP)<\/li>\n<li>MTX<span style=\"color: #000080;\"><strong>\u9ac4\u6ce8\u306f\u7528\u3044\u307e\u305b\u3093<\/strong><\/span><\/li>\n<li>\u305d\u306e\u5f8c\u306b3\u30b3\u30fc\u30b9\u306e\u5927\u91cf\u5316\u5b66\u7642\u6cd5 (CBDCA, TT) \u3068\u672b\u68a2\u8840\u5e79\u7d30\u80de\u6551\u63f4<\/li>\n<li>\u816b\u760d\u5e8a\u306b\u539f\u4f53\u7167\u5c04\u3092\u52a0\u3048\u307e\u3059\u304c\uff0c\u75c5\u5909\u306e\u5e83\u304c\u308a\u3068\u5e74\u9f62\u306b\u3088\u3063\u3066\u6642\u671f\u3068\u7167\u5c04\u9818\u57df\u3092\u5909\u3048\u307e\u3059<\/li>\n<li>3\u6b73\u672a\u6e80\u306f50.4Gy\uff0c\u305d\u308c\u4ee5\u4e0a\u306f54Gy\u3067\u3059\uff0c\u967d\u5b50\u7dda\u306e\u5fdc\u7528\u306f\u8a31\u53ef\u3055\u308c\u3066\u3044\u307e\u3059<\/li>\n<li>\u767b\u933265\u4eba\u306e\u3046\u3061\u306e54\u4eba\u306f3\u6b73\u672a\u6e80\u3067\u3057\u305f<\/li>\n<li>2020\u6642\u70b9\u3067\u306f\u6700\u3082\u826f\u3044\u6cbb\u7642\u6210\u7e3e\u3092\u3060\u3057\u3066\u3044\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\">4\u5e74EFS 37%\uff0c4\u5e74OS 43\uff05<\/span>\u3067\u3057\u305f<\/li>\n<li>4\u5272\u7a0b\u5ea6\u306e\u5b50\u4f9b\u9054\u304c\u9577\u671f\u751f\u5b58\u3067\u304d\u308b\u3068\u8003\u3048\u3066\u3044\u3044\u3067\u3057\u3087\u3046<\/li>\n<li>\u518d\u71c3\u304c\u751f\u3058\u308b\u3068\u3059\u308c\u307091%\u304c2\u5e74\u4ee5\u5185\u306b\u751f\u3058\u307e\u3057\u305f\uff082\u5e74\u518d\u767a\u3057\u306a\u3051\u308c\u3070\u307b\u3068\u3093\u3069\u518d\u767a\u306f\u306a\u3044\u3068\u3044\u3046\u3053\u3068\u3067\u3059\uff09<\/li>\n<\/ul>\n<div class=\"setumei\">\n<h4><span style=\"color: #ff0000;\">IRS-III ATRT protocol<\/span>\u306e\u6982\u8981<\/h4>\n<ul>\n<li>\u307e\u305a\uff0c\u624b\u8853\u6458\u51fa\u3042\u308b\u3044\u306f\u751f\u691c\u3057\u3066INI 1\u907a\u4f1d\u5b50\u306e\u691c\u7d22\u3092\u3057\u307e\u3059<\/li>\n<li><strong><span style=\"color: #000080;\">\u653e\u5c04\u7dda\u6cbb\u7642\u524d\u5316\u5b66\u7642\u6cd5<\/span><\/strong>\u3092\u3057\u307e\u3059<\/li>\n<li><strong><span style=\"color: #003366;\">\u6b8b\u5b58\u816b\u760d\u304c\u3042\u308b\u5834\u5408\u306b\u306f\u518d\u624b\u8853<\/span><\/strong>\u3092\u691c\u8a0e\u3057\u307e\u3059<\/li>\n<li>M0\uff08\u8ee2\u79fb\u304c\u306a\u3044\uff09\u3042\u308b\u3044\u306f<span style=\"color: #000080;\"><strong>\uff13\u6b73\u4ee5\u4e0b\u3067\u306f\u539f\u4f53\u5c40\u6240\u7167\u5c04 conformal irradiation \u3068\u5316\u5b66\u7642\u6cd5<\/strong><\/span>\u3092\u3057\u307e\u3059\u304c\uff0cM+\uff08\u8ee2\u79fb\u304c\u3042\u308b\uff09\u3042\u308b\u3044\u306f<strong><span style=\"color: #003366;\">3\u6b73\u4ee5\u4e0a\u3067\u306f\u8133\u810a\u9ac4\u7167\u5c04\u3068\u5316\u5b66\u7642\u6cd5<\/span><\/strong>\u3092\u884c\u3044\u307e\u3059<\/li>\n<li><strong><span style=\"color: #000080;\">\u653e\u5c04\u7dda\u6cbb\u7642\u5f8c\u5c0e\u5165\u5316\u5b66\u7642\u6cd5<\/span><\/strong>\u3092\u3057\u307e\u3059<\/li>\n<li>\u3055\u3089\u306b\u6b8b\u5b58\u816b\u760d\u304c\u3042\u308b\u4f8b\u3067\u306f<strong><span style=\"color: #000080;\">\u653e\u5c04\u7dda\u5916\u79d1 SRS<\/span><\/strong>\u304c\u884c\u3048\u308b\u304b\u3069\u3046\u304b\u3092\u691c\u8a0e\u3057\u307e\u3059<\/li>\n<li><strong><span style=\"color: #000080;\">\u7dad\u6301\u5316\u5b66\u7642\u6cd5<\/span><\/strong>\u3092\u3057\u307e\u3059<\/li>\n<li>\u8133\u810a\u9ac4\u7167\u5c04\u304c\u306a\u3055\u308c\u306a\u304b\u3063\u305f\u4f8b\u3067\u306e\u307f\u30c9\u30ad\u30bd\u30eb\u30d3\u30b7\u30f3\u7d99\u7d9a\u5316\u5b66\u7642\u6cd5\u3092\u3057\u307e\u3059<\/li>\n<\/ul>\n<h5>WHO 2021\u5e74 \u306e\u75c5\u7406\u8a3a\u65ad\u57fa\u6e96<\/h5>\n<ul>\n<li><span style=\"color: #000080;\"><strong>\u7d44\u7e54\u50cf\u3068INI1\u86cb\u767d\u306e\u6b20\u5931<\/strong><\/span>\u3092\u307f\u3066\u8a3a\u65ad\u3057\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong>INI1\u86cb\u767d<\/strong><\/span>\u767a\u73fe\u306f\u514d\u75ab\u7d44\u7e54\u67d3\u8272\u3067\u8a55\u4fa1\u3059\u308b\u3053\u3068\u304c\u3067\u304d\u307e\u3059<\/li>\n<li>chromosome 22\u306e\u5b8c\u5168\u3042\u308b\u3044\u306f\u90e8\u5206\u7684\u6b20\u5931\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u7d50\u679c\u3068\u3057\u3066\uff0c <span style=\"color: #000080;\"><strong><em>SMARCB1<\/em><\/strong><\/span>gene (<em>hSNF5\/INI1<\/em>)\u306e\u7570\u5e38\uff08<strong><span style=\"color: #000080;\">\u4e0d\u6d3b\u5316<\/span><\/strong>\uff09\u3092\u751f\u3058\u307e\u3059<\/li>\n<li>\u304d\u308f\u3081\u3066\u307e\u308c\u306b\uff0c<em>SMARKA4<\/em> (<em>BRG1<\/em>)\u306e\u5909\u7570\u3092\u6709\u3059\u308b\u3082\u306e\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u3082\u3057\u7d44\u7e54\u8a3a\u65ad\u3067AT\/RT\u3067\u3042\u3063\u3066INI1\u5909\u7570\u304c\u306a\u3044\u5834\u5408\u306f\uff0c<span style=\"color: #000080;\"><strong>CNS embryonal tumor with rhabdoid features<\/strong><\/span>\u3068\u8a18\u8f09\u3057\u307e\u3059<\/li>\n<li>\u514d\u75ab\u67d3\u8272\u3067INI1\u86cb\u767d\u5909\u7570\u304c\u3042\u308a\uff0c<em>SMARCB1<\/em>\u6b20\u5931\u304c\u3042\u3063\u3066\u3082\uff0c\u30e9\u30d6\u30c9\u30a4\u30c9\u69d8\u306e\u7d44\u7e54\u50cf\u898b\u3048\u306a\u3044\u5834\u5408\u306f\uff0c<span style=\"color: #000080;\"><span style=\"color: #000080;\"><strong><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/pediattumor\/pnetembryonaltumor\/\">cribriform neuroepithelial tumor CRINET<\/a>\u00a0\u304b<span style=\"color: #000000;\">\u00a0<strong><a style=\"color: #000000;\" href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/braintumors\/pinealtumor\/\">desmoplastic myxoid tumor of the pineal region, <em>SMARKB1<\/em>-mutant<\/a>\u00a0<\/strong><\/span><\/strong><\/span><\/span><span style=\"color: #000000;\">\u3092\u8003\u3048\u307e\u3059<\/span><\/li>\n<\/ul>\n<h3>AT\/RT\u306e\u305d\u306e\u4ed6\u306e\u77e5\u8b58<\/h3>\n<h4>\u75c5\u7406\u6240\u898b\uff1aEuropean Rhabdoid Registry\u306e\u8a18\u8f09\u3088\u308a<\/h4>\n<p>Macroscopically, AT\/RT resembles medulloblastoma and sPNET. The tumors are soft, pale pink and show areas of hemorrhage as well as necrotic regions. Very commonly <span style=\"color: #ff0000;\"><strong>rhabdoid cells characterized by eosinophilic cytoplasm<\/strong><\/span>, large nuclei with excentric nucleoli and a prominent membrane as well as cytoplasmic eosinophilic inclusion bodies are seen. These diagnostic cells may be grouped in nests close to areas composed of neuroectodermal, mesenchymal or epithelial tissue types. Only about 10 to 15% of AT\/RT consist almost exclusively of rhabdoid cells. AT\/RT exhibit a broad spectrum of immunohistochemical reactions corresponding to the different tissue subtypes. Rhabdoid cells are characterized by expression of vimentin, EMA (epithelial membrane antigen) and cytokeratins, less commonly by SMA (smooth muscle actin). The immunohistochemical demonstration of <strong><span style=\"color: #ff0000;\">lost INI1 protein expression<\/span><\/strong> in the tumor cells is a strong indicator for AT\/RT, however, rare AT\/RT with preserved INI1 expression are also on record.<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-12102 size-full\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/atrt-1.jpg\" alt=\"\" width=\"395\" height=\"299\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/atrt-1.jpg 395w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/atrt-1-300x227.jpg 300w\" sizes=\"auto, (max-width: 395px) 100vw, 395px\" \/><\/p>\n<h4>\u7d30\u5206\u985e<\/h4>\n<ul>\n<li>\u5206\u5b50\u907a\u4f1d\u5b66\u7684\u306b\u306f\u3055\u3089\u306b3\u22124\u3064\u306b\u5206\u985e\u3055\u308c\u307e\u3059<\/li>\n<li>ATRT-TYR\u306f\uff0c\u6700\u3082\u5e7c\u3044\u5b50\uff08\u4e2d\u592e\u502412\u30f6\u6708\uff09\u306b\u767a\u751f\u3057\uff0c\u30c6\u30f3\u30c8\u4e0b\u306b\u591a\u304f\uff0c\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u3067\u5f37\u304f\u5897\u5f37\u3055\u308c\u308b wavy bandlike enhancement\u3068\u3044\u3046\u7279\u5fb4\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>ATRT-SHH1\u306f\u30c6\u30f3\u30c8\u4e0a\uff0cATRT-SHH2\u306f\u30c6\u30f3\u30c8\u4e0b\u306b\u767a\u751f\u3059\u308b\u50be\u5411\u304c\u3042\u308a\uff0c\u4e21\u8005\u3068\u3082\u306bMRI\u6240\u898b\u306f\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u5897\u5f37\u3055\u308c\u306a\u3044\u7279\u5fb4\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>ATRT-MYC\u306f\uff0c\u5e74\u9f62\u5c64\u304c\u9ad8\u304f\uff0c\u30c6\u30f3\u30c8\u4e0a\u306b\u767a\u751f\u3057\uff0c\u5468\u56f2\u8133\u6d6e\u816b\u304c\u6975\u3081\u3066\u9ad8\u5ea6\u3067\u3059<\/li>\n<\/ul>\n<h5 style=\"text-align: left;\"><span style=\"color: #000080;\"><strong>\u5bb6\u65cf\u6027AT\/RT<br \/>\nRhabdoid tumor predisposition syndrome (RTPS)<\/strong><\/span><\/h5>\n<ul>\n<li>\u5bb6\u65cf\u6027\u306e\u3082\u306e\u306f <strong>rhabdoid tumor predisposition syndrome (RTPS)<\/strong>\u3068\u3082\u3044\u3048\u308f\u308c\u307e\u3059<\/li>\n<li><strong><span style=\"color: #000080;\"><i>SMARCA4<\/i><\/span><\/strong>\u304b<span style=\"color: #000080;\"><strong><i>SMARCB1<\/i><\/strong><\/span><em>\u00a0( INI1, hSNF5)\u00a0<\/em>\u907a\u4f1d\u5b50\u306egerm line\u306b\u7570\u5e38\u304c\u3042\u308b\u5bb6\u7cfb\u304c\u77e5\u3089\u308c\u3066\u3044\u307e\u3059<\/li>\n<li>\u5e38\u67d3\u8272\u4f53\u512a\u6027\u907a\u4f1d\u3067\u3059\u304c\u6d78\u900f\u7387\u306f\u4f4e\u3044\u3067\u3059<\/li>\n<li>myoepithelioma\u3084schwannomatosis\u3092\u5408\u4f75\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u3053\u306e\u30bf\u30a4\u30d7\u306eAT\/RT\u306f\uff0c1\u6b73\u672a\u6e80\u306e\u4e73\u5150\u306e\u5c0f\u8133\u306b\u597d\u767a\u3057\u307e\u3059<\/li>\n<li>\u814e\u81d3\u3092\u306f\u3058\u3081\u304b\u3089\u3060\u306e\u3069\u3053\u306b\u3067\u3082\u60aa\u6027\u30e9\u30d6\u30c9\u30a4\u30c9\u816b\u760d\u3092\u5408\u4f75\u3042\u308b\u3044\u306f\u540c\u6642\u767a\u751f\u3059\u308b\u53ef\u80fd\u6027\u304c\u3042\u308a\u307e\u3059<\/li>\n<li><strong><span style=\"color: #ff0000;\">\u3067\u3082\u3057\u304b\u3057\uff0c<i>de novo<\/i> germline <i>SMARCB1<\/i> mutation\u3067\u3059\u304b\u3089<i>\uff0c<\/i>\u5b9f\u969b\u306b\u306f\u307b\u3068\u3093\u3069\u907a\u4f1d\u3057\u306a\u3044\u306e\u3067\u3059<\/span><\/strong><\/li>\n<\/ul>\n<p>Bruggers CS, et al.:\u00a0Clinicopathologic\u00a0comparison\u00a0of\u00a0familial\u00a0versus\u00a0sporadic\u00a0atypical\u00a0teratoid\/rhabdoid\u00a0tumors (AT\/RT) of the central nervous system.Pediatr Blood Cancer.\u00a02011<br \/>\n\u30e6\u30bf\u5927\u5b66\u3067\u306fAT\/RT 20\u4f8b\u306e\u3046\u306112\u4f8b\u304c\u5bb6\u65cf\u6027\u3068\u306e\u8a3a\u65ad\u3067\u3057\u305f\u3002\u3067\u3082\u3053\u306e\u983b\u5ea6\u306f\u9ad8\u3059\u304e\u308b\u3067\u3057\u3087\u3046\u3002\u5bb6\u65cf\u6027\u306eAT\/RT \u767a\u75c7\u4e2d\u592e\u5024\u751f\u5f8c\uff15\u30f6\u6708\uff0c\u751f\u5b58\u671f\u9593\u4e2d\u592e\u50245\u30f6\u6708\u3068\u5b64\u767a\u6027\u306e\u3082\u306e\u3088\u308a\u4e88\u5f8c\u304c\u60aa\u304b\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002<br \/>\n<span lang=\"EN-US\">Bartelheim K, et al.: Improved 6<\/span><span lang=\"EN-US\">\u2010<\/span><span lang=\"EN-US\">year overall survival inAT\/RT\u2013 results of the registry study Rhabdoid 2007.<\/span><span class=\"cit\"><span lang=\"EN-US\">Cancer Med<\/span>. 2016<br \/>\n\u30e8\u30fc\u30ed\u30c3\u30d1\u304b\u3089\u306e\u30c7\u30fc\u30bf\u3067\u3059\u3002germ line mutation\u304c21\u4f8b\u4e2d6\u4f8b(29%)\u306b\u3042\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002<\/span><\/p>\n<h5><span style=\"color: #000080;\">AT\/RT\u306e\u5b66\u8853\u60c5\u5831<\/span><\/h5>\n<h4><strong>ACNS0333\u306e\u7d50\u679c<\/strong>\u00a0: \u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3068\u4e09\u6b21\u5143\u539f\u4f53\u7167\u5c04\u306b\u3088\u308b\u6cbb\u7642<\/h4>\n<\/div>\n<p class=\"heading-title\"><span class=\"authors-list-item \">Reddy AT:\u00a0<\/span>Efficacy of High-Dose Chemotherapy and Three-Dimensional Conformal Radiation for Atypical Teratoid\/Rhabdoid Tumor: A Report From the Children&#8217;s Oncology Group Trial ACNS0333. J Clin Oncol 2020<br \/>\n3\u6b73\u672a\u6e80\u306e54\u4f8b\u306e\u6cbb\u7642\u7d50\u679c\u3067\u3059\u3002\u624b\u8853\u6458\u51fa\u5f8c\u306b\u30012\u30b3\u30fc\u30b9\u306e\u901a\u5e38\u5316\u5b66\u7642\u6cd5\u30013\u30b3\u30fc\u30b9\u306e\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3001\u5c40\u6240\u653e\u5c04\u7dda\u6cbb\u7642\u3067\u6cbb\u7642\u304c\u306a\u3055\u308c\u307e\u3057\u305f\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u306e\u30bf\u30a4\u30df\u30f3\u30b0\u306f\u5e74\u9f62\u3068\u816b\u760d\u306e\u90e8\u4f4d\u306b\u3088\u3063\u3066\u5909\u3048\u308b\u3053\u3068\u304c\u3067\u304d\u307e\u3059\u3002<span style=\"color: #000080;\"><strong>4\u5e74\u7121\u5897\u60aa\u751f\u5b58\u5272\u5408\u306f37%\u3001\u5168\u751f\u5b58\u5272\u5408\u306f43%<\/strong><\/span>\u3067\u3057\u305f\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u306e\u30bf\u30a4\u30df\u30f3\u30b0\u306f\u751f\u5b58\u5272\u5408\u306b\u5f71\u97ff\u3057\u307e\u305b\u3093\u3067\u3057\u305f\u3002\u518d\u767a\u306f\u6cbb\u7642\u5f8c2\u5e74\u4ee5\u5185\u306b\u751f\u3058\u308b\u3068\u306e\u3053\u3068\u3067\u3059\u3002<\/p>\n<div class=\"setumei\">\n<h4>SHH\u3092\u3055\u3089\u306b2\u578b\u306b\u5206\u985e\u3067\u304d\u308b<\/h4>\n<\/div>\n<p>Ho B: Molecular subgrouping of atypicalteratoid\/rhabdoid tumors-a reinvestigation and current consensus.\u00a0<span class=\"labs-docsum-journal-citation full-journal-citation\">Neuro Oncol. 2020<br \/>\n<span style=\"color: #000080;\"><strong>ATRT-SHH (sonic hedgehog), ATRT-TYR, ATRT-MYC<\/strong><span style=\"color: #333333;\">\u306b\u5206\u985e\u3055\u308c\u3066\u304b\u30895\u5e74\uff0c\u4e88\u5f8c\u306a\u3069\u304c\u518d\u691c\u8a3c\u3055\u308c\u307e\u3057\u305f\u3002ATRT-SHH\u306f\uff0c<strong>ATRT-SHH-1,\u00a0ATRT-SHH-2<\/strong>\u306b\u3055\u3089\u306b\u7d30\u5206\u5316\u3055\u308c\u308b\u305d\u3046\u3067\u3059\u3002<\/span><\/span><\/span><\/p>\n<div class=\"setumei\">\n<h4>epigenetic\u306b3\u3064\u306b\u5206\u985e\u3067\u304d\u308b<\/h4>\n<\/div>\n<p>Johann PD, et al.: Atypical\u00a0Teratoid\/Rhabdoid\u00a0Tumors Are Comprised of Three Epigenetic Subgroups with Distinct Enhancer Landscapes.\u00a0Cancer Cell.\u00a02016<br \/>\nTorchia J, et al.:\u00a0Integrated (epi)-Genomic Analyses Identify Subgroup-Specific Therapeutic Targets in CNS\u00a0Rhabdoid\u00a0Tumors.\u00a0Cancer Cell.\u00a02016<br \/>\nTorchia J, et al.:\u00a0Molecular subgroups of\u00a0atypical\u00a0teratoid\u00a0rhabdoid\u00a0tumours in children: an integrated genomic and clinicopathological analysis. Lancet Oncol. 2015<br \/>\nTorcia\u306e\u8ad6\u6587\u3067\u306f259\u4f8b\uff0cJohann\u306e\u8ad6\u6587\u3067\u306f192\u4f8b\u306eAT\/RT\u304c\u89e3\u6790\u3055\u308c\u307e\u3057\u305f\u30023\u3064\u306e\u30b5\u30d6\u30bf\u30a4\u30d7\u306b\u5206\u985e\u3059\u308b\u3053\u3068\u304c\u3067\u304d\u308b\u3068\u306e\u3053\u3068\u3067\u3059\u3002\u85ac\u5264\u611f\u53d7\u6027\u3084\u751f\u5b58\u671f\u9593\u306e\u9577\u77ed\u3082\u7570\u306a\u308b\u3068\u3053\u3068\u3067\u3059\u304c\uff0c\u307e\u3060\u81e8\u5e8a\u5fdc\u7528\u3067\u304d\u308b\u307b\u3069\u306e\u77e5\u898b\u3068\u306f\u306a\u3063\u3066\u3044\u307e\u305b\u3093\u3002<br \/>\n<span style=\"color: #000080;\"><strong>ATRT-SHH (sonic hedgehog), ATRT-TYR (tyrosine), ATRT-MYC (myelocytomatosis oncogene)<\/strong><span style=\"color: #333333;\">\u3068\u547c\u79f0\u3055\u308c\uff0c<\/span><\/span>TYR\u306f\u30c6\u30f3\u30c8\u4e0b\u306b\u591a\u3044\uff0cMYC\u3067\u306f\u816b\u760d\u5468\u8fba\u6d6e\u816b\u304c\u5f37\u3044\uff0cSHH\u3067\u306f\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u5897\u5f37\u6240\u898b\u304c29%\u307b\u3069\u3067\u307f\u3089\u308c\u306a\u3044\uff0c\u306a\u3069\u306e\u753b\u50cf\u4e0a\u306e\u7279\u5fb4\u3082\u3042\u308b\u3088\u3046\u3067\u3059\u3002<\/p>\n<div class=\"page\" title=\"Page 1\">\n<div class=\"layoutArea\">\n<div class=\"column\">\n<h4>cribriform neuroepithelial tumor CRINET<\/h4>\n<p>Johann PD: Cribriform neuroepithelial tumor: molecular characterization of a SMARCB1-deficient non-rhabdoid tumor with favorable long-term outcome. Brain Pathol 2017<br \/>\n20\u4f8b\u306e\u89e3\u6790\u3067\uff0c\u5e74\u9f62\u4e2d\u592e\u502420\u30f6\u6708\u3067\u3057\u305f\u3002\u5168\u751f\u5b58\u671f\u9593\u4e2d\u592e\u5024\u306f125\u30f6\u6708\u306810\u5e74\u3092\u8d85\u3048\u3066\u3044\u307e\u3057\u305f\u3002<\/p>\n<\/div>\n<\/div>\n<\/div>\n<div class=\"setumei\">\n<h4>European Rhabdoid Registry\u304b\u3089\u306e\u5831\u544a\uff1a\u5927\u91cf\u5316\u5b66\u7642\u6cd5<\/h4>\n<p>Benesch M, et al.: High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid\/rhabdoid tumors (AT\/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplantation 49, 370\u2013375, 2014<br \/>\n\u5e74\u9f62\u4e2d\u592e\u5024\u751f\u5f8c21\u30f6\u6708\u306e19\u4eba\u306e\u5b50\u3069\u3082\u304c\u767b\u9332\u3055\u308c\u307e\u3057\u305f\u3002\u8a3a\u65ad\u6642\u306b9\u4eba\u3067\u64ad\u7a2e\u8ee2\u79fb\u306e\u6240\u898b\u304c\u3042\u308a\u307e\u3057\u305f\u3002 \u624b\u8853\u5168\u6458\u51fa\u306f5\u4eba\u3067\u53ef\u80fd\u3067\u3057\u305f\u30026\u30b3\u30fc\u30b9\u7a0b\u5ea6\u306e\u5316\u5b66\u7642\u6cd5\u306e\u5f8c\u306b\u5927\u91cf\u5316\u5b66\u7642\u6cd5 HDCT \u304c\u306a\u3055\u308c\uff0c14\u4eba\u3067\u306f\u653e\u5c04\u7dda\u6cbb\u7642\u304c\u52a0\u3048\u3089\u308c\u307e\u3057\u305f\u3002HDCT\u306e\u524d\u306b6\u4eba\u3067CR\uff0c8\u4eba\u3067PR\uff0c2\u4eba\u3067SD\uff0c2\u4eba\u3067PD\u3068\u306a\u3063\u3066\u3044\u307e\u3057\u305f\u3002\u8ffd\u8de1\u6a5f\u959316\u30f6\u6708\u306714\u4eba\u306b\u816b\u760d\u9032\u884c progression\u304c\u751f\u3058\uff0c2\u5e74\u7121\u5897\u60aa\u751f\u5b58\u5272\u5408\u306f29%\uff0c\u5168\u751f\u5b58\u5272\u5408\u306f50%\u3068\u7b97\u51fa\u3055\u308c\u307e\u3057\u305f\u3002\u6700\u7d42\u8ffd\u8de1\u6642\u70b9\u3067\u306f8\u4eba\u304c\u751f\u5b58\u3057\u3066\u3044\u3066\uff0c11\u4eba (58%) \u304c\u6b7b\u4ea1\u3057\u3066\u3044\u307e\u3059\u3002AT\/RT\u306e\u4e00\u90e8\u306e\u60a3\u5150\u3067\u306f\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3068\u653e\u5c04\u7dda\u6cbb\u7642\u306e\u52b9\u679c\u304c\u3042\u308b\u304b\u3082\u3057\u308c\u306a\u3044\u3068\u3044\u3046\u7d50\u8ad6\u3067\u3059\u3002\u7d50\u8ad6\u3092\u8a73\u3057\u304f\u8aad\u3080\u3068\uff0cHDCT\u306e\u5f8c\u3067\uff13\u5206\u306e1\u306e\u60a3\u5150\u3067\u7d99\u7d9a\u7684\u306a\u5bdb\u89e3\u304c\u5f97\u3089\u308c\u308b\u306e\u3067\u3059\u304c\uff0c\u8ee2\u79fb\u306e\u306a\u3044\u4f8b localized disease\u3068\u5c0e\u5165\u5316\u5b66\u7642\u6cd5\u304c\u594f\u529f\u3057\u305f\u4f8b\u3068\u624b\u8853\u3092\u542b\u3081\u3066\u6b8b\u5b58\u816b\u760d\u304c\u7121\u3044\u3082\u3057\u304f\u306f\u5c0f\u3055\u3044\u4f8b\u3067\u6709\u52b9\u3067\u3042\u308b\u3068\u8a18\u8ff0\u3055\u308c\u3066\u3044\u307e\u3059\u3002<br \/>\n\u300c\u89e3\u8aac\u300d\u4f7f\u7528\u3055\u308c\u305f\u5316\u5b66\u7642\u6cd5\u306f<span style=\"color: #ff0000;\"><strong>Rhabdoid 2007<\/strong><\/span>\u3068<strong><span style=\"color: #ff0000;\">EU-RHAB<\/span><\/strong>\u3067\u3059\u3002Rhabdoid 2007\u306f\uff0cVCD (VCR, CY, ADM), ICE (IFO, CBDCA, VP-16), itMTX\u306e\u30b3\u30f3\u30d3\u30cd\u30fc\u30b7\u30e7\u30f3\u3067\u3059\u3002EU-RHAB\u306f\uff0cADM, ICE, VCA (VCR, CY, actinomycin D), itMTX\u3067\u3059\u3002<span style=\"color: #000080;\"><strong>HDCT\u3067\u306f\uff0cCBDCA, thiotepa<\/strong><\/span>\u304c\u7528\u3044\u3089\u308c\u3066\u3044\u307e\u3059\u30021\u30b3\u30fc\u30b9\u76ee\u306eICE\u306e\u5f8c\u3067stem cell harvest\u304c\u884c\u308f\u308c\u3066\u3044\u307e\u3059\u304b\u3089\uff0c\u5316\u5b66\u7642\u6cd5\u3092\u958b\u59cb\u3059\u308b\u524d\u306b\u3053\u306e\u6cbb\u7642\u8a08\u753b\u3092\u3057\u306a\u3044\u3068\u306a\u308a\u307e\u305b\u3093\u3002\u7b46\u8005\u3089\u304c\u7d50\u8ad6\u3057\u3066\u3044\u308b\u3088\u3046\u306b\uff0c\u5c0e\u5165\u5316\u5b66\u7642\u6cd5\u304c\u594f\u529f\u3057\u306a\u3044\u3068\u3053\u306e\u5927\u91cf\u5316\u5b66\u7642\u6cd5 HDCT\u306b\u8e0f\u307f\u8fbc\u3080\u3079\u304d\u3067\u306f\u306a\u3044\u3068\u306e\u89e3\u91c8\u304c\u59a5\u5f53\u3067\u3057\u3087\u3046\u3002<\/p>\n<\/div>\n<div class=\"setumei\">\n<div>\n<h4>Head Start III\u306e\u6cbb\u7642\u6cd5\u306f\u63a8\u5968\u3067\u304d\u306a\u3044<\/h4>\n<p align=\"left\">Zaky W, et al.: Intensive induction chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue for young children newly-diagnosed with central nervous system atypical teratoid \/rhabdoid tumors: the Head Start III experience. Pediatr Blood Cancer\u00a061: 95-101, 2014<br \/>\nHead Start III\u3068\u3044\u3046\u6cbb\u7642\u8a08\u753b\u306b\u57fa\u3065\u3044\u3066\uff0c19\u4eba\u306eAT\/RT\u306e\u5b50\u3069\u3082\u304c\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3092\u57fa\u672c\u3068\u3057\u3066\u6cbb\u7642\u3055\u308c\u307e\u3057\u305f\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u306f\u539f\u5247\u3068\u3057\u3066\u4f7f\u7528\u305b\u305a\u521d\u671f\u6cbb\u7642\u3092\u958b\u59cb\u3057\u3066\u3044\u307e\u3059\u3002\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u306e\u56de\u5fa9\u3092\u5f85\u3063\u3066\uff0c\u5e74\u9f62\u3092\u8003\u616e\u3057\u3066\uff0c\u8a3a\u65ad\u6642\u306e\u75c5\u5909\u306e\u5e83\u3055\u3092\u9451\u307f\u3066\uff0c\u5c0e\u5165\u7642\u6cd5\u3078\u306e\u53cd\u5fdc\u3092\u307f\u3066\u304b\u3089\u4f7f\u7528\u3055\u308c\u307e\u3057\u305f\u30024\u4eba\u306e\u5b50\u3069\u3082\u3067\u5c0e\u5165\u5316\u5b66\u7642\u6cd5\u3092\u7d42\u3048\u308b\u3053\u3068\u304c\u3067\u304d\u3066\uff0c\u305d\u306e\u5185\u306e3\u4eba\u3067consolidation\u304c\u3067\u304d\u307e\u3057\u305f\u30025\u4eba\u306e\u5b50\u3069\u3082\u304c\u6cbb\u7642\u306e\u526f\u4f5c\u7528\u3067\u6b7b\u4ea1\u3057\u307e\u3057\u305f (5 toxic deaths)\u30023\u5e74\u6642\u70b9\u3067\u306e\u5168\u751f\u5b58\u5272\u5408\u306f26%\u3067\u3057\u305f\u3002\u3053\u306e\u307e\u3068\u3081\u306e\u6642\u70b9\u3067\uff0c3\u5e74\u4ee5\u4e0a\u751f\u5b58\u3057\u3066\u3044\u305f\u5b50\u3069\u3082\u304c4\u4eba (21%) \u3044\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002<br \/>\n\u300c\u89e3\u8aac\u300d\u7d50\u679c\u306f\u71e6\u3005\u305f\u308b\u6210\u7e3e\u3067\u3042\u308a\uff0c\u3053\u308c\u3068\u540c\u3058\u8003\u3048\u65b9\u306e\u6cbb\u7642\u6cd5\u306fAT\/RT\u306b\u7528\u3044\u3089\u308c\u308b\u3079\u304d\u3067\u306f\u306a\u3044\u3068\u8a00\u3048\u307e\u3059\u3002<\/p>\n<h4>\u5c0f\u5150\u8133\u816b\u760d\u5148\u9032\u56fd\u30ab\u30ca\u30c0\u304b\u3089\u306e\u60c5\u5831<\/h4>\n<p align=\"left\">Lafay-Cousin L, et al.: Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 48: 353-359. 2012<br \/>\n\u30ab\u30ca\u30c0\u30671995\u5e74\u304b\u30892007\u5e74\u306e12\u5e74\u9593\u306b\uff0c50\u4eba\u306e\u5b50\u3069\u3082\u304cAT\/RT\u3068\u8a3a\u65ad\u3055\u308c\u307e\u3057\u305f\u3002\u8a3a\u65ad\u5e74\u9f62\u4e2d\u592e\u5024\u306f\u751f\u5f8c16.7\u30f6\u6708\u3067\uff0c52%\u306f\u30c6\u30f3\u30c8\u4e0b\u767a\u751f\u3067\u3057\u305f\u300219\u4eba(38%)\u3067\u8ee2\u79fb\u304c\u3042\u308a\u307e\u3057\u305f\u300210\u4eba\u306f\u7de9\u548c\u30b1\u30a2\u3092\u53d7\u3051\u307e\u3057\u305f\u3002\u6cbb\u7642\u3092\u53d7\u3051\u305f40\u4eba\u306e\u5185\uff0c15\u4eba(30%)\u304c\u5168\u6458\u51fa\u3092\u53d7\u3051\uff0c22\u4eba\u304c\u901a\u5e38\u5316\u5b66\u7642\u6cd5\uff0c18\u4eba\u304c\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3092\u53d7\u3051\u307e\u3057\u305f\u3002\u9ac4\u8154\u5185\u5316\u5b66\u7642\u6cd5\u306f9\u4eba\u3067\u3059\u300215\u4eba\u304c\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\u3044\u307e\u3059\u300230\u4eba\u3067\u816b\u760d\u306e\u9032\u884c\u60aa\u5316\u304c\u3042\u308a\u671f\u9593\u4e2d\u592e\u5024\u306f5.5\u30f6\u6708\u3067\u3057\u305f\u3002<span style=\"color: #ff0000;\"><strong>\u751f\u5b58\u671f\u9593\u4e2d\u592e\u5024\u306f13.5\u30f6\u6708<\/strong><\/span>\uff0c\u6700\u9577\u751f\u5b58\u306f117.5\u30f6\u6708\u3067\uff0c\u5e74\u9f62\uff0c\u816b\u760d\u90e8\u4f4d\uff0c\u64ad\u7a2e\u306f\u751f\u5b58\u671f\u9593\u306b\u95a2\u4fc2\u3042\u308a\u307e\u305b\u3093\u3067\u3057\u305f\u3002<span style=\"color: #ff0000;\"><strong>\u5168\u6458\u51fa\u3067\u304d\u305f\u4f8b\u306e2\u5e74\u751f\u5b58\u5272\u5408\u304c60% (p=0.03)<\/strong>\u00a0<\/span>\u3067\u3042\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002\u5927\u91cf\u5316\u5b66\u7642\u6cd5 CBDCA\/TTP \u3067\u306e\u751f\u5b58\u5272\u5408\u304c\u826f\u3044\u50be\u5411\u304c\u3042\u308a\uff0c\u653e\u5c04\u7dda\u6cbb\u7642 upfront RT\u306f\u5f71\u97ff\u304c\u306a\u304f\uff0c12\u4eba\u306e\u751f\u5b58\u8005\u306e\u3046\u30616\u4eba\u306f\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\u3044\u306a\u304b\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3057\u305f\u3002<br \/>\n\u300c\u89e3\u8aac\u300d<span style=\"color: #ff0000;\"><strong>\u7121\u5897\u60aa\u751f\u5b58\u671f\u9593\u304c\u534a\u5e74\u306b\u6e80\u305f\u306a\u3044<\/strong><\/span>\u3068\u3044\u3046\u3053\u3068\u304c\u78ba\u8a8d\u3055\u308c\u3066\u3044\u307e\u3059\u3002\u5168\u6458\u51fa\u3067\u304d\u305f\u3082\u306e\u306f\u816b\u760d\u306e\u767a\u751f\u90e8\u4f4d\u3068\u5927\u304d\u3055\u304c\u5916\u79d1\u9069\u5fdc\u3068\u306a\u3063\u305f\u3053\u3068\u3092\u793a\u3057\u3066\u3044\u307e\u3059\u306e\u3067\uff0c\u64ad\u7a2e\u3084\u90e8\u4f4d\u304c\u95a2\u4fc2\u306a\u3044\u308f\u3051\u3067\u306f\u3042\u308a\u307e\u305b\u3093\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u305f15\u4eba\u306e\u5185\u306e6\u4eba\u304c\u751f\u5b58\u3057\u3066\u3044\u308b\u3068\u3082\u89e3\u91c8\u3067\u304d\u307e\u3059\u3002<\/p>\n<h4>\u30d5\u30e9\u30f3\u30b9\u304b\u3089\u306e\u60c5\u5831<\/h4>\n<p align=\"left\">Dufour C, et al.: Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system: a multicenter study. Cancer 118: 3812-3821, 2012<br \/>\n1998\u5e74\u304b\u30892008\u5e74\u307e\u3067\u306e\u30d5\u30e9\u30f3\u30b9\u3067\u306e\u7d4c\u9a13\u306e\u307e\u3068\u3081\u3067\u3059\u300258\u4eba\u306e\u5c0f\u5150\u304c\u6cbb\u7642\u3055\u308c\u307e\u3057\u305f\u3002\u8a3a\u65ad\u306e\u5e74\u9f62\u4e2d\u592e\u5024\u306f1.4\u6b73\u3067\uff0c\u751f\u5f8c14\u65e5\u304b\u30898.5\u6b73\u307e\u3067\u3067\u3057\u305f\u3002\u30c6\u30f3\u30c8\u4e0a\u304c26\u4f8b\uff0c\u30c6\u30f3\u30c8\u4e0b\uff08\u5c0f\u8133\u8133\u5e79\uff09\u304c28\u4f8b\u3067\u3057\u305f\u3002INI1 nuclear expression\u306e\u6b20\u640d\u304c50\u4f8b\u4e2d\u306e49\u4f8b(98%)\u306b\u8a8d\u3081\u3089\u308c\uff0cclaudin-6\u304c42\u4f8b\u4e2d37\u4f8b(88%)\u3067\u967d\u6027\u3068\u306a\u308a\uff0c\u03b2-catenin, P53\u306a\u3069\u3082\u534a\u6570\u4ee5\u4e0a\u3067\u967d\u6027\u3067\u3057\u305f\u3002\u521d\u671f\u6cbb\u7642\u306f47\u4eba\u304c\u5316\u5b66\u7642\u6cd5\u3067\uff0c16\u4eba\u304c\u653e\u5c04\u7dda\u6cbb\u7642\u3067\u3057\u305f\u3002<span style=\"color: #ff0000;\"><strong>\u751f\u5b58\u671f\u9593\u4e2d\u592e\u5024\u306f9\u30f6\u6708<\/strong><\/span>\u3068\u304b\u306a\u308a\u53b3\u3057\u3044\u7d50\u679c\u3067\u3057\u305f\u30022\u6b73\u4ee5\u4e0b\uff0c\u64ad\u7a2e\u8ee2\u79fb\u304c\u3042\u3063\u305f\u4f8b\uff0cclaudin-6\u306e\u67d3\u8272\u6027\u304c\u5f37\u3044\u4f8b\u3067\u6709\u610f\u306b\u4e88\u5f8c\u304c\u60aa\u304b\u3063\u305f\u305d\u3046\u3067\u3059\u3002<\/p>\n<h4>\u5927\u91cf\u5316\u5b66\u7642\u6cd5<\/h4>\n<p>Finkelstein-Shechter T, et al.: Atypical Teratoid or Rhabdoid Tumors: Improved Outcome With High-dose Chemotherapy. J Pediatr Hematol Oncol 32: 182\u2013186, 2010<br \/>\n\u30c8\u30ed\u30f3\u30c8\u5b50\u3069\u3082\u75c5\u9662\u304b\u3089\u306e\u5831\u544a\u3067\u3059\u30022003-2008\u5e74\u306b8\u4eba\u306e\u5b50\u3069\u3082\u304cAT\/RT\u3068\u8a3a\u65ad\u3055\u308c\u307e\u3057\u305f\u30025\u4eba\u3067\u3059\u3067\u306b\u9ac4\u6db2\u64ad\u7a2e\u304c\u3042\u3063\u305f\u305d\u3046\u3067\u3059\u3002\u751f\u5f8c1\u30f6\u6708\u30686\u30f6\u6708\u306e\uff12\u4eba\u306e\u4e73\u5150\u306f\u6cbb\u7642\u3092\u53d7\u3051\u305a\uff0c\uff16\u4eba\u306e\u5b50\u3069\u3082\u306b\u624b\u8853\u6cbb\u7642\u3068\u5927\u91cf\u5316\u5b66\u7642\u6cd5\uff08\u5e79\u7d30\u80de\u79fb\u690d\uff09\u304c\u884c\u308f\u308c\u307e\u3057\u305f\u3002\u5c40\u6240\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u305f\u5b50\u3069\u3082\u306f\uff12\u4eba\u3067\u3059\u3002\u7d4c\u904e\u89b3\u5bdf\u671f\u9593\u4e2d\u592e\u502452\u30f6\u6708\u306e\u6bb5\u968e\u3067\uff14\u4eba\u304c\u7121\u5897\u60aa\u751f\u5b58\u3057\u3066\u3044\u307e\u3057\u305f\u304c\uff0c\u5168\u54e1\u3067\u8a8d\u77e5\u6a5f\u80fd\u969c\u5bb3\u304c\u3042\u308a\u307e\u3057\u305f\u3002\u64ad\u7a2e\u304c\u3042\u308b\u72b6\u614b\u3067\u767a\u75c7\u3057\u305f\uff12\u4eba\u3067\u306f\u5316\u5b66\u7642\u6cd5\u306e\u307f\u3067\u9577\u671f\u751f\u5b58\u304c\u5f97\u3089\u308c\u3066\u3044\u307e\u3059\u3002<br \/>\n\u624b\u8853\u5f8c\u306e\u57fa\u672c\u7684\u306a\u5316\u5b66\u7642\u6cd5\u306f3 cycles of VP-16, cyclophosphamide, cisplatin and vincristine\u3067\uff0c\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u306fcarboplatin-thiotepa conditioning regimen\u3067\u3057\u305f\u3002<\/p>\n<h4>AT\/RT\u306e\u60c5\u5831<\/h4>\n<p>Tekautz TM, et al.: Atypical teratoid\/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 23: 1491-1499, 2009<br \/>\n2009\u5e74\u306eJ Clin Oncol\u3068\u3044\u3046\u6a29\u5a01\u306e\u3042\u308b\u96d1\u8a8c\u306b\u8f09\u3063\u305f\u8ad6\u6587\u3067\u3059\u3002 31\u4f8b\u4e2d22\u4f8b\u304c3\u6b73\u672a\u6e80\u3067\u3042\u308a\uff0c\u5168\u54e1\u304c\u624b\u8853\u6458\u51fa\u3092\u53d7\u3051\u306630\u4eba\u304c\u5316\u5b66\u7642\u6cd5\u3092\u53d7\u3051\u307e\u3057\u305f\u30022\u5e74\u5168\u751f\u5b58\u5272\u5408\u306f3\u6b73\u672a\u6e80\u306717%\uff0c3\u6b73\u4ee5\u4e0a\u3067\u306f89%\u3068\u5927\u304d\u306a\u5dee\u304c\u3042\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002<span style=\"color: #ff0000;\"><strong>3\u6b73\u4ee5\u4e0a\u306e\u60a3\u5150\u3067\u306f\u8853\u5f8c\u653e\u5c04\u7dda\u6cbb\u7642\u3068\u5927\u91cf\u306e\u30a2\u30eb\u30ad\u30eb\u5316\u5264\u306e\u6295\u4e0e<\/strong><\/span>\u3067\u6cbb\u308b\u53ef\u80fd\u6027\u304c\u3042\u308b\u3068\u66f8\u304b\u308c\u3066\u3044\u307e\u3059\u3002\u307e\u305f\u5c0f\u6570\u4f8b\u3067\u3059\u304c<span style=\"color: #ff0000;\"><strong>ICE\u5316\u5b66\u7642\u6cd5 (ifosfamide, carboplatin, etoposide)\u00a0<\/strong><\/span>\u304c\u6709\u52b9\u3067\u3042\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002\u4e00\u65b9\uff0c\u591a\u304f\u306e\u60a3\u5150\u304c3\u6b73\u672a\u6e80\u3067\u767a\u75c7\u3057\u307e\u3059\u304c\uff0c\u305d\u306e\u5e74\u9f62\u5c64\u30672\u5e74\u306e\u6642\u70b9\u3067\u306e\u6b7b\u4ea1\u5272\u5408\u306f8\u5272\u3092\u8d85\u3048\u308b\u3068\u7406\u89e3\u3057\u306a\u3051\u308c\u3070\u306a\u308a\u307e\u305b\u3093\u3002\u307e\u305f3\u6b73\u672a\u6e80\u306e\u4f8b\u3067\u306f\u5316\u5b66\u7642\u6cd5\u306e\u6700\u4e2d\u306b\u816b\u760d\u518d\u71c3\uff08\u5897\u5927\u9032\u884c\uff09\u3057\u3066\u3057\u307e\u3046\u3057\uff0c\u8a3a\u65ad\u304b\u3089\u306e\u7121\u5897\u60aa\u671f\u9593\u4e2d\u592e\u5024\u306f4\u30f6\u6708\u306b\u3057\u304b\u306a\u3089\u305a\uff0c\u73fe\u6642\u70b9\u3067\u306f\u6709\u52b9\u306a\u5316\u5b66\u7642\u6cd5\u306e\u30ec\u30b8\u30e1\u30f3\u306f\u898b\u3042\u305f\u3089\u306a\u3044\u3068\u8003\u5bdf\u3055\u308c\u3066\u3044\u307e\u3059\u3002\u9577\u671f\u751f\u5b58\u4f8b\u3067\u306f\u4e73\u5e7c\u5150\u3082\u542b\u3081\u3066up-front radiation therapy\u304c\u3055\u308c\u3066\u3044\u308b\u3068\u306e\u3053\u3068\u3067\u3059\u3002<\/p>\n<h4>AT\/RT\u306e\u60c5\u5831<\/h4>\n<p>Chi SN, et al.: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27: 385-389, 2009<br \/>\n\u4e0b\u306b\u66f8\u3044\u3066\u3042\u308bChi\u3068\u3044\u3046\u5148\u751f\u306e\u5b66\u4f1a\u767a\u8868\u306e\u7d50\u679c\u304c\u8ad6\u6587\u306b\u306a\u3063\u305f\u3082\u306e\u3067\u3059\u30022004\u5e74\u304b\u30892006\u5e74\u307e\u306725\u4f8b\u306eAT\/RT\u306e\u5b50\u4f9b\u304c\u6cbb\u7642\u3055\u308c\u306620\u4eba\u7d50\u679c\u304c\u307e\u3068\u3081\u3089\u308c\u3066\u3044\u307e\u3059\u3002\u5e74\u9f62\u4e2d\u592e\u5024\u306f2\u6b732\u30f6\u6708\uff0c14\u4f8b (70%) \u3067\u306f\u64ad\u7a2e\u304c\u306a\u304f\uff0c11\u4f8b\u3067\u5168\u6458\u624b\u8853\u304c\u3055\u308c\u307e\u3057\u305f\u300215\u4eba\u304c\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\u3044\u307e\u3059\u304c\uff0c\u5e74\u9f62\u3068\u64ad\u7a2e\u304c\u3042\u308b\u304b\u306a\u3044\u304b\u3067\u653e\u5c04\u7dda\u6cbb\u7642\u304c\u9078\u629e\u3055\u308c\uff0c<span style=\"color: #ff0000;\"><strong>\u5c40\u6240\u7167\u5c0454\u30b0\u30ec\u30a4(11\u4eba)\u306e\u307f\u304b\u8133\u810a\u9ac4\u7167\u5c0436\u30b0\u30ec\u30a4(4\u4eba)<\/strong><\/span>\u304c\u52a0\u3048\u3089\u308c\u3066\u3044\u307e\u3059\u30022\u5e74\u7121\u5897\u60aa\u751f\u5b58\u5272\u5408\u306f53\uff05\u3067\uff0c\u5168\u751f\u5b58\u5272\u5408\u306f70%\u3067\u3057\u305f\u3002\u7167\u5c04\u524d\u5316\u5b66\u7642\u6cd5\u306f<span style=\"color: #ff0000;\"><strong>modified IRS-III regimen<\/strong><\/span>\u304c\u4f7f\u7528\u3055\u308c\uff0c\u305d\u306e\u5f8c\u306bchemoradiation induction, postradiation induction, maintenance, continuation, intrathecal therapy\u306a\u3069\u306e\u8907\u96d1\u306a\u6cbb\u7642\u6cd5\u304c\u7d44\u307e\u308c\u3066\u3044\u307e\u3059\u306e\u3067\u539f\u8457\u3092\u8aad\u307e\u306a\u3051\u308c\u3070\u89e3\u308a\u307e\u305b\u3093\u3002 \u8a55\u4fa1\u53ef\u80fd\u3067\u3042\u3063\u305f12\u4f8b\u306e\u7167\u5c04\u524d\u5316\u5b66\u7642\u6cd5\u306e\u6709\u52b9\u6027 (objective response) \u306f58%\u3067\u3057\u305f\u3002\u3053\u306e\u8ad6\u6587\u3092\u8a73\u3057\u304f\u8aad\u3080\u3068\u534a\u6570\u8fd1\u304f\u306e\u5b50\u4f9b\u3067\u6cbb\u308b\u53ef\u80fd\u6027\u304c\u3042\u308b\u6cbb\u7642\u304c\u3067\u304d\u305f\u3068\u3044\u3046\u3053\u3068\u306b\u306a\u308a\u307e\u3059\u3002<\/p>\n<h4>AT\/RT\u306e\u60c5\u5831<\/h4>\n<p align=\"left\">Gardner SL, et al.: Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer 51:235-240, 2008<br \/>\n\u30cb\u30e5\u30fc\u30e8\u30fc\u30af\u5927\u5b66\u304b\u3089\u306e\u5831\u544a\u3067\u3059\u300213\u4eba\u306e\u5b50\u3069\u3082\u304c\uff0cHead Start I\u304b Head Start II\u3068\u3044\u3046\u5316\u5b66\u7642\u6cd5\u306e\u7d44\u307f\u5408\u308f\u305b\u3067\u6cbb\u7642\u3055\u308c\u307e\u3057\u305f\u30025\u30b3\u30fc\u30b9\u306ecisplatin, vincristine, cyclophosphamide, etoposide\u901a\u5e38\u5316\u5b66\u7642\u6cd5\u3002\u3055\u3089\u306b1997\u5e74\u304b\u3089\u306eHS II\u3067\u306f\uff0c\u30e1\u30bd\u30c8\u30ec\u30ad\u30bb\u30fc\u30c8\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u304c\u52a0\u3048\u3089\u308c\u307e\u3057\u305f\u3002\u5730\u56fa\u3081\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u306fcarboplatin,\u00a0thiotepa, etoposide\u3067\uff0c\u81ea\u5bb6\u8840\u6db2\u5e79\u7d30\u80de\u6551\u63f4\u304c\u306a\u3055\u308c\u307e\u3057\u305f(AHPCR)\u3002HS II\u3067\u6cbb\u7642\u3092\u53d7\u3051\u305f\uff17\u4eba\u4e2d3\u4eba (43%) \u306e\u5b50\u3069\u3082\u304c\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u305a\u306b\u751f\u5b58\u3057\u3066\u3044\u305f\u305d\u3046\u3067\u3059\uff08\u6cbb\u7642\u5f8c42\u30f6\u6708\uff0c54\u30f6\u6708\uff0c67\u30f6\u6708\u3067\u3059\uff09\u3002\u3053\u306e\u6cbb\u7642\u65b9\u6cd5\u3092\u771f\u4f3c\u3088\u3046\u306b\u3082\uff0c\u6b8b\u5ff5\u306a\u304c\u3089\u65e5\u672c\u3067\u306ftiotepa\u304c\u4f7f\u3048\u307e\u305b\u3093\u3002<\/p>\n<h4>AT\/RT\u306e\u5b66\u4f1a\u767a\u8868<\/h4>\n<p>Results from a single-arm multiinstitutional study of multiagent intrathecal and systemic chemotherapy with age- and risk-adjusted radiation therapy for childern with newly diagnosed CNS atypical teratoid.\/rhabdoid tumor (DFCI 02-294). Chi S, et al. ISPNO 2008, Chicago<br \/>\n2004\u5e74\u304b\u30892007\u5e74\u306b<span style=\"color: #ff0000;\"><strong>modified IRS-III regimen<\/strong><\/span>\u3068\u3044\u3046\u65b9\u6cd5\u3067\u6cbb\u7642\u3055\u308c\u305f22\u4eba\u306e\u60a3\u5150\u306e\u5831\u544a\u3067\u3059\u3002\u3068\u3066\u3082\u5927\u307e\u304b\u306b\u306f\uff0c\uff16\u9031\u9593\u306e\u5c0e\u5165\u5316\u5b66\u7642\u6cd5\u306e\u5f8c\u3067\uff0c\u653e\u5c04\u7dda\u6cbb\u7642\uff08\u5316\u5b66\u7642\u6cd5\u4f75\u7528\uff09\u304c\u3055\u308c\u3066\uff0c\u3055\u3089\u306b\u5730\u56fa\u3081\u79d1\u5b66\u7642\u6cd5\u304c\uff16\u9031\u9593\uff0c\u7dad\u6301\u5316\u5b66\u7642\u6cd5\u304c 26\u9031\u9593\uff0c\u8ffd\u52a0\u306e\u5316\u5b66\u7642\u6cd5\u3092\u3055\u3089\u306b\uff16\u9031\u9593\u3059\u308b\u65b9\u6cd5\u3067\u3059\u3002\u5236\u304c\u3093\u5264\u306e\u9ac4\u8154\u5185\u6295\u4e0e\u3082\u884c\u308f\u308c\u307e\u3057\u305f\u3002\u8ee2\u79fb\u306e\u306a\u3044\u4f8b(M0)\u3067\u306f\u5c40\u6240\u7167\u5c04confomal RT\u309254\u30b0\u30ec\u30a4\u3057\u3066\u307e\u3059\u3002\uff13\u6b73\u4ee5\u4e0a\u3067\u8ee2\u79fb\uff08\u64ad\u7a2e\uff09\u306e\u3042\u308b\u4f8b\u3067\u306f36\u30b0\u30ec\u30a4\u306e\u8133\u810a\u9ac4\u7167\u5c04\u304c\u3055\u308c\u3066\u3044\u307e\u3059\u3002\u5e74\u4ee4\u306e\u4e2d\u592e\u5024\u306f2.5\u6b73\uff0c\u30c6\u30f3\u30c8\u4e0a\u304c12\u4f8b\u3067\uff0c\u30c6\u30f3\u30c8\u4e0b\u304c10\u4f8b\u3067\u3057\u305f\u3002\u534a\u5206\u306e\u60a3\u5150\u3067\u304a\u3088\u305d\u306e\u5168\u6458\u51fa\u304c\u306a\u3055\u308c\u3066\u3044\u307e\u3059\u300216\u4f8b(73%)\u3067\u8ee2\u79fb\u304c\u306a\u3044M0\u3068\u3044\u3046stage\u3067\u3057\u305f\u3002\u4e88\u5b9a\u306e\u6cbb\u7642\u304c\u3059\u3079\u3066\u5b8c\u9042\u3067\u304d\u305f\u306e\u306f22\u4eba\u4e2d\u306e12\u4eba\u3067\u3042\u308a\uff0c\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u305f\u306e\u306f17\u4eba\u3067\u3057\u305f\u3002\u653e\u5c04\u7dda\u524d\u306e\u5316\u5b66\u7642\u6cd5\u594f\u529f\u7387(CR+PR)\u306f62%\u3068\u3044\u3046\u9ad8\u3044\u6570\u5b57\u3067\u3059\u3002\uff12\u5e74\u306e\u6642\u70b9\u3067\u306e\u7121\u5897\u60aa\u751f\u5b58\u5272\u5408\u306f48%\u3067\uff0c\u5168\u751f\u5b58\u5272\u5408\u306f67%\u3067\u3057\u305f\u3002\u5316\u5b66\u7642\u6cd5\u306e\u526f\u4f5c\u7528\u306f\u5f53\u7136\u5f37\u3044\u3082\u306e\u3067\uff0c\u5316\u5b66\u7642\u6cd5\u6b7b\u304c\uff11\u4f8b\u3042\u3063\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002<br \/>\n\u3053\u306e\u5831\u544a\u3092\u307f\u308b\u9650\u308a\uff0c\u307b\u3068\u3093\u3069\u6551\u3046\u3053\u3068\u306e\u3067\u304d\u306a\u3044\u3068\u3055\u308c\u305fAT\/RT\u306e\u5b50\u4f9b\u305f\u3061\u306b\u3082\u751f\u5b58\u306e\u6a5f\u4f1a\u306f\u3042\u308b\u3068\u601d\u3048\u307e\u3059\u3002<\/p>\n<h4>AT\/RT\u306e\u5b66\u4f1a\u767a\u8868<\/h4>\n<p>CNS ATYPICAL TERATOID RHABDOID TUMOR (ATRT) IN CHILDREN LESS THAN 36 MONTHS: A CANADIAN PEDIATRIC BRAIN TUMOR CONSORTIUM (CPBTC) EXPERIENCE. Lafay-Cousin L, et al. ISPNO 2008, Chicago<br \/>\n\u30ab\u30ca\u30c0\u3067\u306e\u8abf\u67fb\u3067\u3059\u3002\uff13\u6b73\u4ee5\u4e0b\u306e\u8133\u816b\u760d\u306e\u5b50\u4f9b531\u4eba\u306e\u3046\u3061\u306e24\u4eba(4.5%)\u304cAT\/RT\u3067\u3057\u305f\u3002\u5e73\u5747\u8a3a\u65ad\u6642\u5e74\u4ee4\u306f12.6\u30f6\u6708\u3067\u3059\u3002\u30c6\u30f3\u30c8\u4e0a\u304c33%\uff0c\u30c6\u30f3\u30c8\u4e0b\u304c58%\uff0c \u810a\u9ac4\u304c8%\u3067\u3059\u300224\u4eba\u4e2d\u306e10\u4eba(42%)\u3067\u8a3a\u65ad\u6642\u304b\u3089\u3059\u3067\u306b\u64ad\u7a2e\u8ee2\u79fb\u304c\u3042\u308a\u307e\u3057\u305f\u3002\uff18\u4eba\u304c\u624b\u8853\u3060\u3051\uff0c7\u4eba\u304c\u5316\u5b66\u7642\u6cd5\u306e\u307f\u3092\u53d7\u3051\uff0c\uff19\u4eba\u304c\u653e\u5c04\u7dda\u5316\u5b66\u7642\u6cd5\u3092\u53d7\u3051\u3066\u3044\u307e\u3059\u3002\u816b\u760d\u304c\u6cbb\u307e\u3063\u3066\u3044\u305f\u7121\u5897\u60aa\u751f\u5b58\u671f\u9593\u4e2d\u592e\u5024\u306f9\u30f6\u6708\uff0c\u5916\u79d1\u624b\u8853\u306e\u3042\u3068\u3067\u8ffd\u52a0\u6cbb\u7642\u3092\u53d7\u3051\u305f16\u4eba\u306e\u751f\u5b58\u671f\u9593\u4e2d\u592e\u5024\u306f16\u30f6\u6708\u3067\u3057\u305f\u3002\uff11\u5e74\u751f\u5b58\u5272\u540850%\uff0c\uff12\u5e74\u751f\u5b58\u5272\u5408\u306f19%\u3067\u3059\u300290\uff05\u4ee5\u4e0a\u306e\u5207\u9664\u3092\u53d7\u3051\u305f\u5b50\u4f9b\u306e\u751f\u5b58\u671f\u9593\u304c\u306a\u304c\u304b\u3063\u305f\u3088\u3046\u3067\u3059\u304c\uff0c\u653e\u5c04\u7dda\u6cbb\u7642\u3068\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u306f\u4e88\u5f8c\u306b\u5bc4\u4e0e\u3057\u306a\u304b\u3063\u305f\u3068\u3057\u3066\u3044\u307e\u3059\u3002\u7d50\u679c\u7684\u306b\uff13\u4eba\u306e\u5b50\u4f9b\u304c\u751f\u5b58\u3057\u3066\u3044\u3066\uff0c\u305d\u306e\u751f\u5b58\u671f\u9593\u306f\uff0c20, 21, 78\u30f6\u6708\u3060\u305d\u3046\u3067\uff0c\u3053\u308c\u3089\u306e\u5b50\u4f9b\u306f90\uff05\u4ee5\u4e0a\u306e\u5207\u9664\u304c\u3055\u308c\u3066\uff0c\uff11\u4f8b\u306f\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\uff0c\uff12\u4f8b\u306f\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3092\u53d7\u3051\u3066\u3044\u307e\u3057\u305f\u3002<\/p>\n<h4>AT\/RT\u306e\u5b66\u4f1a\u767a\u8868<\/h4>\n<p>German pediatric atypical teratoid\/thabdoid tumors: Interim analysis of the ATRT-CNS study and retrospecitve analysis. Peters O, et al. ISPNO 2008, Chicago<br \/>\n\u30c9\u30a4\u30c4\u304b\u3089\u306e19\u75c7\u4f8b\u306e\u6cbb\u7642\u5831\u544a\u3067\u3059\u3002\uff11\u4f8b\u3067\u5b8c\u5168\u6458\u51fa\uff0c\uff15\u4f8b\u3067\u4e9c\u5168\u6458\uff0c\uff19\u4f8b\u3067\u90e8\u5206\u6458\u51fa\uff0c\uff14\u4f8b\u3067\u751f\u691c\u8853\u304c\u306a\u3055\u308c\u307e\u3057\u305f\u3002\u624b\u8853\u5f8c\u306b\uff0c\uff12\u30b3\u30fc\u30b9\u306e\u5c0e\u5165\u5316\u5b66\u7642\u6cd5\uff08doxorubicin, dactinomycin, cisplatin, vincristine, methotrexate)\u3092\u884c\u3063\u305f\u5f8c\u306b\uff0c54Gy\u306e\u5c40\u6240\u7167\u5c04\u3068carboplatin\u306e\u4f75\u7528\u304c\u3055\u308c\uff0c\u3055\u3089\u306b\u6700\u521d\u306e\u5316\u5b66\u7642\u6cd5\u3068\u304a\u306a\u3058\u3082\u306e\u304c\uff11\u30b3\u30fc\u30b9\u6295\u4e0e\u3055\u308c\u307e\u3057\u305f\u3002 \u5730\u56fa\u3081\u7642\u6cd5\u3068\u3057\u3066\uff16\u30b3\u30fc\u30b9\u306eCCNU, cisplatin, vincristine, methotrexate)\u304c\u8ffd\u52a0\u3055\u308c\u3066\u3044\u307e\u3059\u3002\u7121\u5897\u60aa\u304a\u3088\u3073\u5168\u751f\u5b58\u5272\u5408\u306f79%\u3067\u3042\u308a\uff0c\u751f\u5b58\u671f\u9593\u306e\u5e73\u5747\u5024\u306f50\u30f6\u6708\u3068\u5831\u544a\u3055\u308c\u307e\u3057\u305f\u3002 \u6700\u521d\u306e\uff12\u30b3\u30fc\u30b9\u306e\u5316\u5b66\u7642\u6cd5\u306e\u594f\u529f\u7387\u306f\uff0c1 continued complete response, 2 complete response, 13 partial response, 3 stable disease\u3068\u304b\u306a\u308a\u9ad8\u3044\u3082\u306e\u3067\u3059\u3002\u6709\u5bb3\u4e8b\u8c61\u306e\u969b\u7acb\u3063\u305f\u3082\u306e\u306f\u7c98\u819c\u969c\u5bb3\u3068\u611f\u67d3\u75c7\u3067\u3042\u3063\u305f\u3068\u8a18\u9332\u3055\u308c\u3066\u3044\u307e\u3059\u304c\uff0c\u76ee\u7acb\u3063\u305f\u8133\u810a\u9ac4\u969c\u5bb3\u306f\u5831\u544a\u304c\u306a\u304b\u3063\u305f\u3068\u3044\u3046\u3053\u3068\u3067\u3059\u3002\u7d50\u8ad6\u3068\u3057\u3066\uff0cAT\/RT\u306f\u5316\u5b66\u7642\u6cd5\u306b\u611f\u53d7\u6027\u306e\u3042\u308b\u816b\u760d\u3067\u3042\u308b\u304c\uff0c\u5316\u5b66\u7642\u6cd5\u306e\u6709\u5bb3\u4e8b\u8c61\u306f\u304b\u306a\u308a\u5f37\u3044\u3068\u3055\u308c\u3066\u3044\u307e\u3059\u3002<\/p>\n<h4>AT\/RT\u306e\u7dcf\u8ad6<\/h4>\n<p>Squire SE, et al. Atypical teratoid\/rhabdoid tumor: the controversy behind radiaiton therapy. J Neurooncol 81: 97-111, 2007<\/p>\n<ul>\n<li>AT\/RT\u306f\u5c0f\u5150\u8133\u816b\u760d\u306e2-3%<\/li>\n<li>\u9ac4\u82bd\u816b\u306b\u5fdc\u7528\u3055\u308c\u308b\u6cbb\u7642\u3067\u306f\u6cbb\u3089\u306a\u3044<\/li>\n<li>\u624b\u8853\u6458\u51fa\u3068\u5316\u5b66\u7642\u6cd5\u306e\u307f\u3067\u306f\u304b\u306a\u308a\u306e\u4f8b\u3067\u5c40\u6240\u518d\u71c3\u304c\u751f\u3058\u3066\u3044\u308b<\/li>\n<li>\u9577\u671f\u751f\u5b58\u4f8b\u306f\uff0c\u521d\u671f\u6cbb\u7642\u306b\u5927\u91cf\u5316\u5b66\u7642\u6cd5\u3068\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u5fdc\u7528\u3057\u305f\u6642\u306b\u671f\u5f85\u3067\u304d\u308b<\/li>\n<li>vimentin, EMA, SMA\u306e\u967d\u6027\u7387\u304c\u9ad8\u3044<\/li>\n<li>\u7b2c22\u67d3\u8272\u4f5322q11 \u306e\u7570\u5e38\u304c\u9ad8\u7387\u306b\u3042\u308b<\/li>\n<li>complete resection\u304coverall survival\u306b\u6709\u610f\u306b\u5f71\u97ff\u3059\u308b<\/li>\n<li>Intergroup Rhabdomyosarcoma III Study (IRS-III) \u306e\u30ac\u30a4\u30c9\u30e9\u30a4\u30f3\u3067\u6cbb\u7642\u3055\u308c\u9577\u671f\u751f\u5b58\u3057\u305f\u4f8b\u304c\u6563\u898b\u3055\u308c\u308b<\/li>\n<li>high-dose platinum-based or alkylator-based chmotherapy\u304c\u5fdc\u7528\u3055\u308c\u308b\u3053\u3068\u304c\u591a\u3044<\/li>\n<li>\u653e\u5c04\u7dda\u6cbb\u7642\u306e\u57fa\u672c\u7684\u5f79\u5272\u306f\u5c40\u6240\u518d\u767a\u3092\u9632\u3050\u3053\u3068\u3067\u3042\u308b\u304c\uff0c\u8133\u810a\u9ac4\u7167\u5c04\u3092\u3068\u308a\u3044\u308c\u308bregimen\u304c\u591a\u3044<\/li>\n<li>\u8a3a\u65ad\u5f8c\uff0c\uff13\u6b73\u672a\u6e80\u306e\u4f4e\u5e74\u9f62\u5150\u3067\u3042\u308d\u3046\u3068\u3082\u3067\u304d\u308c\u3070\u65e9\u671f\u306e\u653e\u5c04\u7dda\u6cbb\u7642\u304c\u5fc5\u8981\u3067\u3042\u308d\u3046<\/li>\n<li>\u4f4e\u5e74\u9f62\u5150\u306e\u305f\u3081\u306b\u5c40\u6240\u653e\u5c04\u7dda\u6cbb\u7642\u306b\u6b62\u3081\u3056\u308b\u3092\u5f97\u306a\u3044\u3068\u3044\u3046\u8003\u3048\u65b9\u3082\u3042\u308b<\/li>\n<li>\u518d\u767a\u306e1\/3\u304b\u30891\/2\u306f\u9ac4\u6db2\u64ad\u7a2e\u3067\u3042\u308b<\/li>\n<li>\u816b\u760d\u5e8a\u306b\u306f50-55Gy\u7a0b\u5ea6\uff0c\u8133\u810a\u9ac4\u306b\u306f23-30Gy\u3092\u7528\u3044\u308b\u3053\u3068\u304c\u591a\u3044<\/li>\n<li>photon-IMRT\u306e\u6709\u7528\u6027\u306f\u308f\u304b\u3089\u306a\u3044<\/li>\n<li>radiosurgery (SRS) \u3067\u5c40\u6240\u3092\u6cbb\u7642\u3067\u304d\u308c\u3070\u6709\u52b9\u3067\u3042\u308d\u3046<\/li>\n<\/ul>\n<h4>\u6700\u521d\u306e\u8ad6\u6587<\/h4>\n<p><span style=\"color: #000080;\"><strong>1985\u5e74\u306bBriner<\/strong><\/span>\u306b\u3088\u3063\u3066\u306f\u3058\u3081\u3066\u8a18\u8f09\u3055\u308c\u307e\u3057\u305f\u3002Briner, J., et al.: Malignant small cell tumor of the brain with intermediate filaments &#8211; a case of a primary cerebral rhabdoid tumor.\u00a0 Pediatric Pathology 3 : 117-118, 1985<br \/>\n\u5f53\u6642\u306fINI-1\u304c\u77e5\u3089\u308c\u3066\u3044\u307e\u305b\u3093\u3067\u3057\u305f\u306e\u3067\u3001\u5149\u9855\u75c5\u7406\u3067\u306e\u5f62\u614b\u8a3a\u65ad\u3067\u3057\u305f\u304b\u3089\uff0crhabdoid cell\u304c\u76ee\u7acb\u305f\u306a\u3044\u3068AT\/RT\u3068\u8a3a\u65ad\u3055\u308c\u307e\u305b\u3093\u30021990\u5e74\u4ee3\u3067\u306f\u3053\u306e\u8a3a\u65ad\u57fa\u6e96\u306b\u3088\u3063\u3066\u306e\u6b7b\u4ea1\u5272\u5408\u306f100\uff05\u306b\u8fd1\u3044\uff0c\u4f59\u547d\u306f6\u30f6\u6708\u4ee5\u5185\u3068\u8a00\u308f\u308c\u305f\u3053\u3068\u3082\u3042\u308a\u307e\u3057\u305f\u3002\u3069\u3093\u306a\u6cbb\u7642\u3092\u3057\u3066\u3082\u6b7b\u4ea1\u3059\u308b\u60aa\u6027\u8133\u816b\u760d\u3067\u3042\u308b\u3068\u6349\u3048\u3089\u308c\u305f\u306e\u3067\u3059\u3002\u305d\u306e\u5f8c\u306bINI1\u5909\u7570\u306b\u3088\u3063\u3066\u306e\u8a3a\u65ad\u304c\u512a\u5148\u3055\u308c\u308b\u3088\u3046\u306b\u306a\u308a\uff0crhabdoid cell\u304c\u512a\u4f4d\u3067\u306a\u304f\u3066\u3082AT\/RT\u3068\u75c5\u7406\u8a3a\u65ad\u3055\u308c\u308b\u3088\u3046\u306b\u306a\u308a\u307e\u3057\u305f\u3002\u304b\u3064\u3066\u306e\u816b\u760d\u7fa4\u3068\u306f\u3053\u3068\u306a\u3063\u305fcohort\u3067\u3042\u308b\u53ef\u80fd\u6027\u3092\u542b\u3080\uff0c\u3053\u306e\u8a3a\u65ad\u57fa\u6e96\u306e\u5909\u5316\u306b\u3088\u3063\u3066\uff0c\u9577\u671f\u751f\u5b58\u4f8b\u304c20%\u3092\u8d8a\u3048\u308b\u3068\u3044\u3046\u5909\u5316\u304c\u751f\u3058\u307e\u3057\u305f\u3002\u3082\u3061\u308d\u3093\u753b\u50cf\u8a3a\u65ad\uff0c\u624b\u8853\uff0c\u5316\u5b66\u7642\u6cd5\u306e\u9032\u6b69\u3082\u751f\u5b58\u5272\u5408\u306e\u5411\u4e0a\u306b\u5bc4\u4e0e\u3057\u3066\u3044\u307e\u3059\u3002<i><\/i><\/p>\n<\/div>\n<h4>\u6587\u732e<\/h4>\n<ul>\n<li>Benesch M, et al.: High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid\/rhabdoid tumors (AT\/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplantation 49, 370\u2013375, 2014<\/li>\n<li>Briner, J., et al.: Malignant small cell tumor of the brain with intermediate filaments &#8211; a case of a primary cerebral rhabdoid tumor.\u00a0 Pediatric Pathology 3 : 117-118, 1985<\/li>\n<li>Chi SN, et al.: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27: 385-389, 2009<\/li>\n<li>Dufour C, et al.: Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system: a multicenter study. Cancer 118: 3812-3821<\/li>\n<li>Finkelstein-Shechter T, et al.: Atypical Teratoid or Rhabdoid Tumors: Improved Outcome With High-dose Chemotherapy. J Pediatr Hematol Oncol 32: 182\u2013186, 2010<\/li>\n<li>Gardner SL, et al.: Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer 51:235-240, 2008<\/li>\n<li>Lafay-Cousin L, et al.: Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 48: 353-359. 2012<\/li>\n<li>Squire SE, et al. Atypical teratoid\/rhabdoid tumor: the controversy behind radiaiton therapy. J Neurooncol 81: 97-111, 2007<\/li>\n<li>Tekautz TM, et al.: Atypical teratoid\/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 23: 1491-1499, 2009<\/li>\n<li>Zaky W, et al.: Intensive induction chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue for young children newly-diagnosed with central nervous system atypical teratoid \/rhabdoid tumors: the Head Start III experience. Pediatr Blood Cancer\u00a061: 95-101, 2014<\/li>\n<\/ul>\n<\/div>\n<div class=\"setumei\">\n<hr \/>\n<p style=\"text-align: center;\"><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/2007_0530_102526-IMGP6417.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-2186\" title=\"2007_0530_102526-IMGP6417\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/2007_0530_102526-IMGP6417.jpg\" alt=\"\" width=\"600\" height=\"404\" \/><\/a><\/p>\n<p>&nbsp;<\/p>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>\u975e\u5b9a\u578b\u5947\u5f62\u816b\u69d8\uff0f\u30e9\u30d6\u30c9\u30a4\u30c9\u816b\u760d embryonal tumor WHO grade 4 AT\/RT\u306f\u65e5\u672c\u8a9e\u306e\u9069\u5207\u306a\u8a33\u304c\u3042\u308a\u307e\u305b\u3093\u304b\u3089\uff0c\u305d\u306e\u307e\u307eAT\/RT\uff08\u30a8\u30fc\u30c6\u30a3\u30fc\u30fb\u30a2\u30fc\u30eb\u30c6\u30a3\u30fc\uff09\u3068\u547c\u3070\u308c\u307e\u3059 \u80ce\u5150\u671f\u306e\u7d30\u80de\u304b\u3089\u767a\u751f\u3059\u308b\u3068 [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[5],"tags":[],"class_list":["post-158","post","type-post","status-publish","format-standard","hentry","category-pediattumor"],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/158","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/comments?post=158"}],"version-history":[{"count":73,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/158\/revisions"}],"predecessor-version":[{"id":21863,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/158\/revisions\/21863"}],"wp:attachment":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/media?parent=158"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/categories?post=158"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/tags?post=158"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}