{"id":8100,"date":"2015-09-22T10:11:06","date_gmt":"2015-09-22T01:11:06","guid":{"rendered":"\/sawamura\/?p=8100"},"modified":"2023-03-06T17:57:06","modified_gmt":"2023-03-06T08:57:06","slug":"nf-1","status":"publish","type":"post","link":"https:\/\/plaza.umin.ac.jp\/sawamura\/braintumors\/nf-1\/","title":{"rendered":"\u795e\u7d4c\u7dda\u7dad\u816b\u75c7 1\u578b  NF-1"},"content":{"rendered":"<div class=\"setumei\">\n<h5>NF-1 neurofibromatosis type 1<\/h5>\n<ul>\n<li>\u89aa\u304b\u3089\u5b50\u306b\u907a\u4f1d\uff08\u5e38\u67d3\u8272\u4f53\u512a\u6027\u907a\u4f1d\u75be\u60a3\uff09\u3059\u308b\u3053\u3068\u3082\u3042\u308a\u307e\u3059\u304c\uff0c<span style=\"color: #000080;\"><strong>\u534a\u5206\u4ee5\u4e0a\u306e\u60a3\u8005\u3055\u3093\u306f\u907a\u4f1d\u3067\u306f\u3042\u308a\u307e\u305b\u3093<\/strong><\/span><\/li>\n<li><strong><span class=\"setumei\" style=\"color: #ff0000;\">\uff11\u578b (NF-1 \u30a8\u30cc\u30a8\u30d5\u30ef\u30f3)\u00a0<span style=\"color: #000000;\">\u306f\uff0c<\/span><span style=\"color: #000080;\">\u5168\u8eab\u306e\u76ae\u819a\u306b\u305f\u304f\u3055\u3093\u306e\u5c0f\u3055\u306a\u816b\u760d\uff08\u672b\u68a2\u795e\u7d4c\u7dda\u7dad\u816b\uff09\u3068\u8336\u8272\u306e\u30b7\u30df\u306e\u3088\u3046\u306a\u3082\u306e\uff08\u30ab\u30d5\u30a7\u30aa\u30ec\u6591\uff09<\/span><\/span><\/strong>\u304c\u3067\u304d\u308b\u75c5\u6c17\u3067\u3059<\/li>\n<li>\uff11\u578b NF-2 \u306f\u304b\u3064\u3066\u30d5\u30a9\u30f3\u30ec\u30c3\u30af\u30ea\u30f3\u30b0\u30fb\u30cf\u30a6\u30bc\u30f3\u6c0f\u75c5\u3068\u3057\u3066\u77e5\u3089\u308c\u3066\u3044\u307e\u3057\u305f<\/li>\n<li>\u7b2c17\u756a\u3081\u306e\u67d3\u8272\u4f53\u306b\u3042\u308b<span style=\"color: #000080;\"><strong><em>NF1<\/em>\u907a\u4f1d\u5b50\u306e\u5909\u7570<\/strong><\/span>\u3067neurofibromin\u3068\u3044\u3046\u86cb\u767d\u304c\u6b63\u5e38\u306b\u50cd\u304b\u306a\u304f\u306a\u3063\u3066\u8d77\u3053\u308b\u75c5\u6c17\u3067\u3059<\/li>\n<li><strong><span style=\"color: #ff0000;\">\uff12\u578b (NF-2 \u30a8\u30cc\u30a8\u30d5\u30c4\u30fc)<\/span><\/strong><strong><span style=\"color: #ff0000;\"><span style=\"color: #000000;\">\u00a0\u306f\uff0c<\/span><span style=\"color: #000080;\">\u8133\u795e\u7d4c\u3068\u810a\u9ac4\u795e\u7d4c\u306b\u795e\u7d4c\u9798\u816b\u3084\u9ac4\u819c\u816b<\/span><\/span><\/strong>\u304c\u305f\u304f\u3055\u3093\u3067\u304d\u308b\u75c5\u6c17\u3067\u3059<\/li>\n<li>\u7b2c22\u756a\u3081\u306e\u67d3\u8272\u4f5322q\u306b\u3042\u308bNF2\u3068\u3044\u3046\u907a\u4f1d\u5b50\u306e\u5909\u7570\u3067merlin\u3068\u3044\u3046\u86cb\u767d\u304c\u6b63\u5e38\u306b\u50cd\u304b\u306a\u304f\u306a\u3063\u3066\u8d77\u3053\u308b\u75c5\u6c17\u3067\u3059<\/li>\n<li>\u4e21\u65b9\u3068\u3082\u306b\u3042\u308b\u7a0b\u5ea6\u75c5\u6c17\u304c\u91cd\u3044\u3068\u533b\u7642\u8cbb\u304c\u88dc\u52a9\u3055\u308c\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong>\uff11\u578b\u306e\u60a3\u8005\u3055\u3093\uff083,000\u4eba\u306b\u4e00\u4eba\uff09\u306e\u65b9\u304c\uff12\u578b\uff08\u6570\u4e07\u4eba\u306b\u4e00\u4eba\uff09<\/strong><\/span>\u3088\u308a\u304b\u306a\u308a\u591a\u3044\u3067\u3059<\/li>\n<li>\uff11\u578b\u3067\u6cbb\u7642\u3057\u306a\u3051\u308c\u3070\u306a\u3089\u306a\u3044\u8133\u816b\u760d\u306f\u3081\u305a\u3089\u3057\u304f\uff0c\uff12\u578b\u306e\u8133\u816b\u760d\u306f\u6cbb\u7642\u3092\u5fc5\u8981\u3068\u3059\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li><strong><span style=\"color: #ff0000;\">\u3044\u305a\u308c\u3082\u826f\u6027\u816b\u760d\u3067\u3059<\/span><\/strong><span style=\"color: #000000;\">\u304b\u3089\u6cbb\u7642\u3092\u3042\u308f\u3066\u308b\u5fc5\u8981\u306f\u3042\u308a\u307e\u305b\u3093<\/span><\/li>\n<li><strong><span style=\"color: #000080;\">\u305f\u304f\u3055\u3093\u816b\u760d\u304c\u3042\u308b\u3068\u3044\u3063\u3066\u3082\u6cbb\u7642\u3092\u3057\u306a\u3051\u308c\u3070\u306a\u3089\u306a\u3044\u304b\u3069\u3046\u304b\u304b\u306a\u308a\u614e\u91cd\u306b\u8003\u3048\u308b\u3053\u3068\u304c\u5927\u5207\u3067\u3059<\/span><\/strong><\/li>\n<li>\u3060\u3044\u305f\u3044\u304c\u826f\u6027\u816b\u760d\u306a\u306e\u3067\u4f55\u3082\u3057\u306a\u3044\u3067\u304a\u3044\u3066\u3082\u75c7\u72b6\u304c\u60aa\u304f\u306a\u3089\u306a\u3044\u3053\u3068\u3082\u3042\u308b\u306e\u3067\u3059<\/li>\n<li>2\u578b\u306b\u306f\u305f\u304f\u3055\u3093\u306e\u8133\u810a\u9ac4\u816b\u760d\u304c\u3067\u304d\u308b\u306e\u3067\u3059\u304c\uff0c\u305d\u306e\u3046\u3061\u306e<span style=\"color: #000080;\"><strong>\u3069\u306e\u816b\u760d\u304c\u5927\u304d\u304f\u306a\u308b\u304b\u306f\u4e88\u6e2c\u304c\u3067\u304d\u307e\u305b\u3093<\/strong><\/span><\/li>\n<li>\uff12\u578b\u306b\u3067\u304d\u308b\u4e21\u5074\u306e\u8074\u795e\u7d4c\u816b\u760d\u306e\u6cbb\u7642\u306f\u3068\u3066\u3082\u96e3\u3057\u3044\u306e\u3067\uff0c\u666e\u901a\u306e\u8133\u5916\u79d1\u533b\u306e\u5148\u751f\u306b\u306f\u6cbb\u7642\u3067\u304d\u307e\u305b\u3093\u304b\u3089\u305d\u308c\u306b\u8a73\u3057\u3044\u5c02\u9580\u5bb6\u306b\u76f8\u8ac7\u3057\u307e\u3057\u3087\u3046<\/li>\n<li><span style=\"color: #000080;\"><strong>\u5931\u660e\u3059\u308b\u5371\u967a\u306f1\u578b\u306b\u30822\u578b\u306b\u3082\u3042\u308a\u307e\u3059<\/strong><\/span>\u304b\u3089\uff0c\u305d\u308c\u306b\u6c17\u3065\u304b\u306a\u3044\u533b\u5e2b\u306b\u306f\u3053\u306e\u75c5\u6c17\u3092\u307f\u308b\u529b\u91cf\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<li>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u306b\u306f\uff0c<span style=\"color: #000080;\"><strong>\u6cbb\u7642\u85ac\u30b3\u30bb\u30eb\u30b4\uff08\u30bb\u30eb\u30e1\u30c1\u30cb\u30d6\uff09<\/strong><\/span>\u304c\u4f7f\u7528\u3067\u304d\u308b\u3088\u3046\u306b\u306a\u308a\u307e\u3057\u305f<\/li>\n<\/ul>\n<hr \/>\n<h5><span style=\"color: #000080;\"><span class=\"style3\">\u795e\u7d4c\u7dda\u7dad\u816b\u75c7\u306b\u306f<span style=\"color: #ff0000;\">\u533b\u7642\u8cbb<\/span><\/span><strong><span style=\"color: #ff0000;\">\u306e\u88dc\u52a9\u304c\u3042\u308a\u307e\u3059<\/span><\/strong><\/span><\/h5>\n<\/div>\n<hr \/>\n<h5><a href=\"\/sawamura\/braintumors\/nf-2\/\">\u795e\u7d4c\u7dda\u7dad\u816b\u75c72\u578b NF-2\u306f\u4ed6\u306e\u30da\u30fc\u30b8\u306b\u8a73\u3057\u3044\u306e\u3067\u3053\u3053\u3092\u30af\u30ea\u30c3\u30af<\/a><\/h5>\n<h1>\u795e\u7d4c\u7dda\u7dad\u816b\u75c7\uff11\u578b<\/h1>\n<div class=\"setumei\">\n<hr \/>\n<h4><strong>\u795e\u7d4c\u7dda\u7dad\u816b\u75c71\u578b NF-1\u3068\u8133\u816b\u760d<\/strong><\/h4>\n<ul>\n<li>\u3055\u307e\u3056\u307e\u306a\u795e\u7d4c\u81a0\u816b\uff08\u30b0\u30ea\u30aa\u30fc\u30de\uff09\u3068\u3044\u3046\u306e\u304c\u3067\u304d\u307e\u3059\u3002<\/li>\n<li>NF-1\u60a3\u8005\u3055\u3093\u306e20%\u306b\u30b0\u30ea\u30aa\u30fc\u30de\u304c\u767a\u751f\u3059\u308b\u3068\u3082\u8a00\u308f\u308c\u307e\u3059<\/li>\n<li>\u3057\u304b\u3057\uff0c\u591a\u304f\u306f\u30b0\u30ea\u30aa\u30fc\u30de\u3068\u3044\u3046\u3088\u308a\u904e\u8aa4\u816b hamartomatous glioma \u3042\u308b\u3044\u306f\u6bdb\u69d8\u7d30\u80de\u6027\u661f\u7d30\u80de\u816b\u3067\u3059<\/li>\n<li>\u307b\u3068\u3093\u3069\u304c\u6cbb\u7642\u306e\u5fc5\u8981\u304c\u306a\u3044\u3082\u306e\u3067\uff0c\u307b\u3063\u3066\u304a\u3044\u3066\u3082\u5927\u304d\u304f\u306a\u3089\u306a\u3044\u3082\u306e\u304c\u591a\u3044\u3067\u3059<\/li>\n<li>\u5b50\u4f9b\u306b\u898b\u3089\u308c\u308b<strong><span style=\"color: #000080;\"><a href=\"\/sawamura\/pediattumor\/opticpathwaypil\/\">\u8996\u795e\u7d4c\u81a0\u816b<\/a><\/span><\/strong>\u306f\u983b\u5ea6\u306e\u9ad8\u3044\u3082\u306e\u3067\uff0cNF-1\u306e4%\u306b\u767a\u751f\u3057\u307e\u3059<\/li>\n<li>\u4e0b\u306e\u56f3\u306e\u3088\u3046\u306b\u773c\u7aa9\u306e\u4e2d\u306e\u8996\u795e\u7d4c\u304c\u816b\u760d\u306b\u306a\u308b\u3053\u3068\u3082\u3042\u308a\u307e\u3059\u3057\uff0c\u8996\u795e\u7d4c\u4ea4\u53c9\u3068\u3044\u3063\u3066\u4e21\u65b9\u306e\u8996\u795e\u7d4c\u304c\u5408\u308f\u3055\u3063\u305f\u5834\u6240\u306b\u3067\u304d\u308b\u3082\u306e\u3082\u3042\u308a\u307e\u3059\u3002\u8996\u795e\u7d4c\u81a0\u816b\u306f\u3068\u3066\u3082\u5c0f\u3055\u3044\u5b50\u4f9b\u306b\u3067\u304d\u3066\u8996\u529b\u304c\u4e0b\u304c\u308a\u307e\u3059\u304c\uff0c\u624b\u8853\u3067\u6458\u51fa\u3059\u308b\u3053\u3068\u306f\u3067\u304d\u307e\u305b\u3093\u3002\u3067\u3082\uff0c\u3068\u3063\u3066\u3082\u3086\u3063\u304f\u308a\u5927\u304d\u304f\u306a\u308a\uff0c\u3042\u308b\u7a0b\u5ea6\u306e\u5e74\u9f62\u306b\u306a\u308b\u3068\u5927\u304d\u304f\u306a\u308b\u306e\u304c\u6b62\u307e\u3063\u3066\u3057\u307e\u3044\u307e\u3059\u3002\u624b\u8853\u4ee5\u5916\u306e\u6cbb\u7642\u6cd5\u306f<a href=\"..\/pediattumor\/pilo\/\">\u6bdb\u69d8\u7d30\u80de\u6027\u661f\u7d30\u80de\u816b\uff08\u30af\u30ea\u30c3\u30af\uff09<\/a>\u306e\u3068\u3053\u308d\u306b\u66f8\u3044\u3066\u3042\u308a\u307e\u3059\u304b\u3089\u8aad\u3093\u3067\u4e0b\u3055\u3044\u3002<br \/>\n<a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/07\/neurofibromatosis\/NF1optilgli1.png\"><img loading=\"lazy\" decoding=\"async\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/07\/neurofibromatosis\/NF1optilgli1.png\" alt=\"\" width=\"202\" height=\"199\" \/><\/a><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/07\/neurofibromatosis\/NF1optilgli2.png\"><img loading=\"lazy\" decoding=\"async\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/07\/neurofibromatosis\/NF1optilgli2.png\" alt=\"\" width=\"192\" height=\"198\" \/><\/a><\/li>\n<li>\u6bdb\u69d8\u7d30\u80de\u6027\u661f\u7d30\u80de\u816b\u306f\uff0c\u8996\u5e8a\u4e0b\u90e8\u3084\u5c0f\u8133\u306b\u3082\u3067\u304d\u307e\u3059<\/li>\n<li>\u5c0f\u8133\u306b\u306f\u3073\u307e\u3093\u6027\u661f\u7d30\u80de\u816b\u3082\u3067\u304d\u307e\u3059\u304c\uff0c\u305d\u306e\u4e2d\u306b\u306f\u60aa\u6027\u5ea6\u306e\u9ad8\u3044\u3082\u306e\u3082\u3042\u308a\u307e\u3059<\/li>\n<li>\u5927\u8133\u306b\u3082\u3055\u307e\u3056\u307e\u306a\u795e\u7d4c\u81a0\u816b\u304c\u3067\u304d\u307e\u3059<\/li>\n<li>\u81a0\u82bd\u816b\u306e\u3088\u3046\u306b\u60aa\u6027\u5ea6\u306e\u9ad8\u3044\u3082\u306e\u3084\u4e4f\u7a81\u8d77\u81a0\u816b\u306e\u3088\u3046\u306b\u6cbb\u308b\u3082\u306e\u3082\u3042\u308a\u307e\u3059<\/li>\n<\/ul>\n<p><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo2.png\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-15442\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo2-258x300.png\" alt=\"\" width=\"258\" height=\"300\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo2-258x300.png 258w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo2.png 315w\" sizes=\"auto, (max-width: 258px) 100vw, 258px\" \/><\/a><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-15443\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo1-271x300.png\" alt=\"\" width=\"271\" height=\"300\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo1-271x300.png 271w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NF1oligo1.png 323w\" sizes=\"auto, (max-width: 271px) 100vw, 271px\" \/><\/p>\n<p>\u5c0f\u5150\u306b\u3067\u304d\u305f\u53f3\u524d\u982d\u8449\u306e\u4e4f\u7a81\u8d77\u81a0\u816b\u30b0\u30ec\u30fc\u30c92\u3067\u3059\u3002\u53f3\u306f\u8853\u5f8c\u306e\u5199\u771f\u3067\uff0c\u624b\u8853\u3060\u3051\u3067\u6cbb\u308a\u307e\u3057\u305f\u3002<\/p>\n<h5>\u8133\u5e79\u90e8\u30b0\u30ea\u30aa\u30fc\u30de<\/h5>\n<ul>\n<li>NF-1\u3067\u306f\u8133\u5e79\u90e8\u306b\u3082\u305f\u304f\u3055\u3093\u306e\u75c5\u5909\u304c\u307f\u3089\u308c\u307e\u3059\uff0c<\/li>\n<li><a title=\"NF-1\u306b\u5408\u4f75\u3059\u308b\u8133\u5e79\u90e8\u30b0\u30ea\u30aa\u30fc\u30de\" href=\"\/sawamura\/braintumors\/glioma\/nfibrainstem\/\"><span style=\"color: #000080;\"><strong>\u7279\u306b\u6c17\u3092\u3064\u3051\u306a\u3051\u308c\u3070\u306a\u3089\u306a\u3044\u306e\u306f\uff0c\u5f90\u3005\u306b\u5927\u304d\u304f\u306a\u3063\u3066\u304f\u308b\u8133\u5e79\u90e8\u30b0\u30ea\u30aa\u30fc\u30de brainstem glioma\u3067<\/strong><\/span><\/a><span style=\"color: #000080;\"><strong><a style=\"color: #000080;\" title=\"NF-1\u306b\u5408\u4f75\u3059\u308b\u8133\u5e79\u90e8\u30b0\u30ea\u30aa\u30fc\u30de\" href=\"\/sawamura\/braintumors\/glioma\/nfibrainstem\/\">\u3059\uff08\u3053\u3053\u3092\u30af\u30ea\u30c3\u30af\u3059\u308b\u3068\u5177\u4f53\u4f8b\u304c\u898b\u308c\u307e\u3059\uff09<\/a><\/strong><\/span><\/li>\n<li>\u5927\u304d\u304f\u306a\u3063\u3066\u3082\u6cbb\u7642\u306e\u5fc5\u8981\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<\/ul>\n<h4><strong>NF-1\u3067\u8133\u816b\u760d\u3068\u9593\u9055\u3063\u3066\u3044\u306f\u3044\u3051\u306a\u3044\u8133\u306e\u75c5\u5909<\/strong><\/h4>\n<ul>\n<li>\u8133\u306e\u3068\u3053\u308d\u3069\u3053\u308d\u306b\uff0cT2\/\u30d5\u30ec\u30a2\u3067\u4f55\u304b\u7279\u5b9a\u3067\u304d\u306a\u3044\u9ad8\u4fe1\u53f7\u304c\u307f\u3048\u307e\u3059 (<span style=\"color: #ff0000;\"><strong>UBO\u00a0unidentified bright object<\/strong><strong>s<\/strong><\/span> \u3042\u308b\u3044\u306fFASI focal area of signal intensity\u3068\u3044\u3044\u307e\u3059\uff09<\/li>\n<li>\u75c5\u7406\u5b66\u7684\u306b\u306f\uff0c<span style=\"color: #000080;\"><strong>\u9ac4\u9798\u7a7a\u6d1e\u5316(myelin vacuolization<\/strong><\/span>, altered myelination)\uff0c<span style=\"color: #000080;\"><strong>\u904e\u5f62\u6210\u6027\u30b0\u30ea\u30aa\u30fc\u30b7\u30b9(hyperplastic gliosis)<\/strong><\/span>\u3044\u308f\u308c\u308b\u3082\u306e\u3067\u3059<\/li>\n<li>\u904e\u8aa4\u816b(hamartoma)\uff0c\u7570\u5f62\u6210(dysplasia, heterotopia)\uff0c\u8133\u5ba4\u4e0a\u8863\u4e0b\u7d50\u7bc0(subependymal glial fibrillary nodue)\u306a\u3069\u3082\u3042\u308a\u307e\u3059<\/li>\n<li>MRI\u3067\u816b\u760d\u306e\u3088\u3046\u306b\u307f\u3048\u307e\u3059\u304c\u305d\u3046\u3067\u306f\u3042\u308a\u307e\u305b\u3093\u3002T2\u5f37\u8abf\u753b\u50cf\u3067\u767d\u304f\u898b\u3048\u3066\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u9020\u5f71\u3055\u308c\u306a\u3044\u306e\u304c\u4e00\u822c\u7684\u306a\u7279\u5fb4\u3067\u3059<\/li>\n<\/ul>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-full wp-image-11325\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NFbrainstem1.png\" alt=\"\" width=\"263\" height=\"266\" \/><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NFbrainstem2.png\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-full wp-image-11326\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/NFbrainstem2.png\" alt=\"\" width=\"272\" height=\"280\" \/><\/a><\/p>\n<p>\u8133\u5e79\u90e8\u306eUBO\u3067\u3059\uff0c\u5927\u304d\u304f\u306a\u308b\u3068\u8133\u5e79\u90e8\u795e\u7d4c\u81a0\u816b\u306b\u9593\u9055\u3048\u3089\u308c\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-5039\" title=\"nf1gliosis\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1gliosis1-287x300.jpg\" alt=\"\" width=\"287\" height=\"300\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1gliosis1-287x300.jpg 287w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1gliosis1.jpg 308w\" sizes=\"auto, (max-width: 287px) 100vw, 287px\" \/><br \/>\nNF-1\u306b\u898b\u3089\u308c\u308b\u5178\u578b\u7684\u306aUBO\u3067\u3059\u3002\u767d\u304f\u306b\u3058\u3080\u3088\u3046\u306b\u898b\u3048\u308b\u3068\u3053\u308d\u304c\uff0c\u4e21\u5074\u306e\u5927\u8133\u57fa\u5e95\u6838\u304b\u3089\u8996\u5e8a\u306b\u6563\u5728\u3057\u3066\u3044\u307e\u3059\u3002\u8133\u5e79\u90e8\u306b\u3082\u540c\u6642\u306b\u898b\u3089\u308c\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059\u3002\u3082\u3061\u308d\u3093\u6cbb\u7642\u306e\u5fc5\u7528\u306f\u3042\u308a\u307e\u305b\u3093\u3002<\/p>\n<h4>\u8133\u5e79\u90e8\u30b0\u30ea\u30aa\u30fc\u30de\u3068\u9593\u9055\u3048\u305d\u3046\u306a\u3082\u306e<\/h4>\n<p><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-17101\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1-300x289.jpg\" alt=\"\" width=\"300\" height=\"289\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1-300x289.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1-550x530.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1-768x740.jpg 768w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm1.jpg 1133w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-17102\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2-300x295.jpg\" alt=\"\" width=\"300\" height=\"295\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2-300x295.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2-550x541.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2-768x755.jpg 768w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2015\/09\/neurofibromatosisnm2.jpg 1134w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><\/p>\n<p>3\u6b73\u306e\u6642\u306b\uff0cMRI\u3067\u8133\u5e79\u90e8\u304b\u3089\u5c0f\u8133\u306e\u30b0\u30ea\u30aa\u30fc\u30de\u3092\u7591\u308f\u308c\u3066\u53d7\u8a3a\u3057\u307e\u3057\u305f\u3002\u6a4b\u304c\u816b\u308c\u3066\u53f3\u5c0f\u8133\u306b\u3082\u30b0\u30ea\u30aa\u30fc\u30de\u306e\u3088\u3046\u306a\u767d\u3044\u5f71\u304c\u3042\u308a\u307e\u3059\u3002\u3067\u3082\u3053\u308c\u306f\uff0cNF-1\u306eUBO\u306e\u5927\u304d\u306a\u3082\u306e\u3067\u3059\u3002\u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de\u3068\u8aa4\u8a3a\u3055\u308c\u3066\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\u3057\u307e\u3063\u305f\u5b50\u4f9b\u3082\u307f\u305f\u3053\u3068\u304c\u3042\u308a\u307e\u3059\u3002\u6cbb\u7642\u3057\u306a\u304f\u3066\u3082\u7e2e\u5c0f\u3057\u3066\u3044\u304f\u306e\u3067\uff0c\u4f55\u3082\u3057\u306a\u3044\u3067\u7d4c\u904e\u89b3\u5bdf\u3057\u307e\u3059\u3002\u53f3\u5074\u306eMRI\u306f13\u5e74\u5f8c\u306e\u3082\u306e\u3067\u3059\u3002\u307b\u3068\u3093\u3069\u6d88\u5931\u3057\u3066\u3044\u307e\u3059\u3002<\/p>\n<hr \/>\n<h4><strong><span style=\"color: #000080;\">NF-1\u3068NF-2\u306e\u6c34\u982d\u75c7<\/span><\/strong><\/h4>\n<ul>\n<li>NF1\u3067\u306f\uff0c\u591a\u304f\u306e\u5834\u5408\u306f\u904e\u5f62\u6210\u6027\u30b0\u30ea\u30aa\u30fc\u30b7\u30b9\u3067<strong><span style=\"color: #000080;\">\u4e2d\u8133\u6c34\u9053\u3068\u3044\u3046\u9ac4\u6db2\u306e\u6d41\u308c\u9053\u304c\u3064\u307e\u3063\u3066\u6c34\u982d\u75c7\uff08\u4e2d\u8133\u6c34\u9053\u9589\u585e\uff09<\/span><\/strong>\u306b\u306a\u308a\u307e\u3059\u3002\u3053\u308c\u306f\u6025\u306b\u75c7\u72b6\u3092\u51fa\u3059\u3053\u3068\u304c\u306a\u304f\u3066\u6162\u6027\u306e\u982d\u75db\u3068\u304b\u3092\u751f\u3058\u308b\u505c\u6b62\u6027\u6c34\u982d\u75c7\u306b\u306a\u308a\u3084\u3059\u3044\u3067\u3059\u3002\u6cbb\u7642\u306f\uff0c\u5185\u8996\u93e1\u3092\u4f7f\u3063\u3066\u9ac4\u6db2\u306e\u901a\u308a\u9053\u3092\u3042\u3051\u308b\u7b2c\uff13\u8133\u5ba4\u958b\u7a93\u8853\u3068\u3044\u3046\u306e\u3092\u3057\u305f\u3089\u6cbb\u308a\u307e\u3059\u3002<\/li>\n<li><strong><span style=\"color: #ff0000;\">NF-2\u306b\u751f\u3058\u308b\u73fe\u8c61\u3067\u3059\u304c\uff0c\u8133\u816b\u760d\u304c\u591a\u767a\u3059\u308b\u3053\u3068\u306b\u3088\u3063\u3066\u9ac4\u6db2\u5438\u53ce\u969c\u5bb3\u304c\u751f\u3058\u3066\uff0c\u4ea4\u901a\u6027\u6c34\u982d\u75c7\u306b\u306a\u308a\u307e\u3059<\/span><\/strong>\u3002\u3053\u306e\u5834\u5408\u306b\uff0c\u7279\u306b\u6c17\u3092\u4ed8\u3051\u306a\u3051\u308c\u3070\u306a\u3089\u306a\u3044\u306e\u304c\u8133\u5ba4\u304c\u5927\u304d\u304f\u306a\u3089\u306a\u3044 (slit ventricle) \u306e\u3067\uff0c\u6c34\u982d\u75c7\u3067\u306f\u306a\u3044\u3068\u601d\u3063\u3066\u3057\u307e\u3046\u3053\u3068\u3067\u3059\u3002\u773c\u5e95\u691c\u67fb\u3092\u3059\u308b\u3068\uff0c\u3046\u3063\u8840\u4e73\u982d\u304b\u3042\u308b\u3044\u306f\u4e73\u982d\u840e\u7e2e\u306e\u6240\u898b\u304c\u3042\u308a\u307e\u3059\u3002\u6025\u3044\u3067\uff0c\u8133\u5ba4\u8179\u8154\u30b7\u30e3\u30f3\u30c8\u3092\u3057\u306a\u3051\u308c\u3070\u306a\u308a\u307e\u305b\u3093\u304c\uff0c\u5b9a\u4f4d\u8133\u624b\u8853\u3067\u3057\u304b\u8133\u5ba4\u7aef\u3092\u523a\u5165\u3059\u308b\u3053\u3068\u304c\u3067\u304d\u307e\u305b\u3093\u3002 \u305d\u3057\u306a\u3044\u3068\u5931\u660e\u3057\u307e\u3059\u3002<\/li>\n<\/ul>\n<\/div>\n<div class=\"setumei\">\n<h4><strong>NF-1\u306e\u672b\u68a2\u795e\u7d4c\u306e\u75c5\u6c17<\/strong><\/h4>\n<ul>\n<li>\u307b\u3068\u3093\u3069\u306e\u3082\u306e\u306f\u75c7\u72b6\u3092\u51fa\u3057\u307e\u305b\u3093<\/li>\n<li>\u57fa\u672c\u7684\u306b\u306f\u591a\u5c11\u5927\u304d\u304f\u306a\u3063\u3066\u3082\u307b\u3063\u3066\u304a\u304d\u307e\u3059<\/li>\n<li>\u307e\u308c\u306b\u56f0\u3063\u305f\u75c7\u72b6\u3092\u51fa\u3057\u307e\u3059<\/li>\n<li>\u810a\u9ac4\u795e\u7d4c\u306e\u6839\u5143\uff08\u810a\u690e\uff09\u306b\u795e\u7d4c\u9798\u816b\u304c\u3067\u304d\u3066\u810a\u9ac4\u3092\u5727\u8feb\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u00a0<strong><span style=\"color: #000080;\">plexiform neurofibroma<\/span><\/strong>\u3068\u3044\u3046\u306e\u306f\u3042\u308b\u7bc4\u56f2\uff08\u4f8b\u3048\u3070\u53f3\u306e\u9996\u3042\u305f\u308a\u3068\u304b\uff09\u306b\u8513\u72b6\u306e\u7dda\u7dad\u816b\u304c\u305f\u304f\u3055\u3093\u3067\u304d\u3066\u305d\u308c\u304c\u5927\u304d\u306a\u56fa\u307e\u308a\u3092\u4f5c\u3063\u3066\u304f\u308b\u3082\u306e\u3092\u3044\u3044\u307e\u3059\u304c\uff0c\u8133\u306b\u306f\u3067\u304d\u307e\u305b\u3093<\/li>\n<li>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u306f\u3068\u3066\u3082\u5e83\u304f\u5e83\u304c\u308b\u306e\u3067\uff0c\u624b\u8853\u3067\u5168\u90e8\u53d6\u308b\u3053\u3068\u304c\u3067\u304d\u307e\u305b\u3093<\/li>\n<li>NF-1\u306e\u60a3\u8005\u3055\u3093\u304c\u4e00\u756a\u56f0\u308b\u306e\u306f\u8513\u72b6\u795e\u7d4c\u7e4a\u7dad\u7a2e\u306e\u5897\u5927\u3067\u3059<\/li>\n<li>\u9854\u9762\u3084\u4f53\u5e79\u306b\u8513\u72b6\u795e\u7d4c\u9798\u816b\u304c\u3067\u304d\u3066\u5909\u5f62\u3057\u305f\u308a\uff0c\u75db\u307f\u304c\u51fa\u3066\u56f0\u308a\u307e\u3059<\/li>\n<li>2020\u5e74\u6642\u70b9\u3067\uff0c\u30bb\u30eb\u30e1\u30c1\u30cb\u30d6\u3068\u3044\u3046\u85ac\u5264\u304c\u671f\u5f85\u3055\u308c\u3066\u3044\u307e\u3059\u304c\uff0c\u65e5\u672c\u3067\u306f\u4f7f\u3048\u307e\u305b\u3093<\/li>\n<li>\u4e2d\u5e74\u304b\u3089\u9ad8\u9f62\u8005\u306b\u306a\u308b\u30685%\u304f\u3089\u3044\uff0c\u3042\u308b\u3044\u306f1000\u4eba\u306b\u4e00\u4eba\u304f\u3089\u3044\u306eNF-1\u60a3\u8005\u3055\u3093\u3067\u795e\u7d4c\u7dda\u7dad\u816b\u304c\u60aa\u6027\u816b\u760d\uff08<a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/braintumors\/mpnst\/\">\u60aa\u6027\u672b\u68a2\u795e\u7d4c\u9798\u816b\u00a0<strong><span style=\"color: #000080;\">MPNST<\/span><\/strong>\u00a0malignant peripheral nerve sheath tumor<\/a>, fibrous sarcoma \u7dda\u7dad\u8089\u816b\uff09\u306b\u306a\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u3084\uff0c\u975e\u5b9a\u578b\u7684\u795e\u7d4c\u7dda\u7dad\u816b atypical neurofibroma\u306f\uff0cMPNST\u3078\u3068\u5897\u60aa\u3057\u3084\u3059\u3044\u306e\u3067\u6ce8\u610f\u3067\u3059<\/li>\n<li>FDG\u30da\u30c3\u30c8\u3067\u53d6\u308a\u8fbc\u307f\u304c\u3042\u308b\uff0c\u75db\u307f\u304c\u5f37\u3044\u5897\u5927\u6027\u306e\u816b\u7624\u3067\uff0c\u75c5\u7406\u50cf\u304catypical neurofibroma\u3067\u3042\u308c\u3070\uff0c\u304b\u306a\u308a\u7a4d\u6975\u7684\u306b\u5b8c\u5168\u6458\u51fa\u3059\u308b\u5fc5\u8981\u304c\u3042\u308a\u307e\u3059<\/li>\n<\/ul>\n<h4>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u306e\u6cbb\u7642\u85ac\uff1a<span style=\"color: #ff0000;\"><strong>\u30b3\u30bb\u30eb\u30b4<\/strong><\/span>\uff08\u30bb\u30eb\u30e1\u30c1\u30cb\u30d6 MEK\u963b\u5bb3\u5264\uff09<\/h4>\n<ul>\n<li>\u65e5\u672c\u3067\u3082\uff0c3\u6b73\u304b\u308918\u6b73\u307e\u3067\u306e<span style=\"color: #ff0000;\"><strong>\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b<\/strong><\/span>\u306b\u306f\uff0c\u30b3\u30bb\u30eb\u30b4\u304c\u4f7f\u7528\u3067\u304d\u308b\u3088\u3046\u306b\u306a\u308a\u307e\u3057\u305f<\/li>\n<li>\u5f37\u3044\u75db\u307f\u3084\u9854\u304c\u5927\u304d\u304f\u5909\u5f62\u3059\u308b\u306a\u3069\u306e\u9032\u884c\u4f8b\u306b\u6295\u4e0e\u3067\u304d\u307e\u3059<\/li>\n<li>1\u65e5\uff12\u56de\u7a7a\u8179\u6642\u306b\u98f2\u307f\u307e\u3059\uff0c10mg, 25mg\u30ab\u30d7\u30bb\u30eb\u304c\u3042\u308a\u307e\u3059\uff0c\u4e0a\u9650\u306f50mg\u3067\u3059<\/li>\n<li>\u6295\u4e0e\u671f\u95932\u5e74\u304f\u3089\u3044\u306e\u7814\u7a76\u3067\u306f\uff0c\u816b\u760d\u306e\u5927\u304d\u304f\u306a\u308b\u306e\u304c\u6b62\u307e\u3063\u305f\u308a\u5c0f\u3055\u304f\u306a\u3063\u305f\u308a\u3059\u308b\u4f8b\u304c66%\u304f\u3089\u3044\u3067\u307f\u3089\u308c\u308b\u305d\u3046\u3067\u3059<\/li>\n<li>\u816b\u760d\u304c\u6d88\u3048\u308b\u3053\u3068\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<\/ul>\n<h4>\u91cd\u8981\u306a\u60c5\u5831\uff08<span style=\"color: #ff0000;\">NF-1\u306e\u5b50\u4f9b\u306e\u8996\u795e\u7d4c\u81a0\u816b\u3078\u306e\u653e\u5c04\u7dda\u6cbb\u7642\u306f\u5371\u967a<\/span>\uff09<\/h4>\n<p>Sharif S, Ferner R, Jillian M. Birch, James E. Gillespie, H. Rao Gattamaneni, Michael E. Baser, D. Gareth R. Evans: Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy. J Clin Oncol: 2570-2575, 2006<br \/>\n\u30a4\u30ae\u30ea\u30b9\u304b\u3089\u306e\u5831\u544a\u3067\u3059\u300218\u4eba\u306eNF-1\u306e\u60a3\u8005\u3055\u3093\u304c\u8996\u795e\u7d4c\u81a0\u816b\u306e\u305f\u3081\u306b\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u307e\u3057\u305f\u3002\u305d\u306e\u5185\u306e9\u4eba (50%) \u306b2\u6b21\u816b\u760d\u304c\u767a\u751f\u3057\uff0c\u5b50\u4f9b\u306e\u6642\u306b\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u3066\u3044\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u306a\u304b\u3063\u305f40\u4eba\u306e\u60a3\u8005\u3055\u3093\u3067\u306f8\u4eba (20%)\u306b2\u6b21\u816b\u760d\u304c\u767a\u751f\u3057\u307e\u3057\u305f\u30022\u6b21\u816b\u760d\u3068\u306f\uff0c15\u4f8b\u306e\u795e\u7d4c\u81a0\u816b\u30686\u4f8b\u306eMPNST\uff08\u60aa\u6027\u672b\u68a2\u795e\u7d4c\u9798\u816b\uff09\u3067\u3059\u30022\u6b21\u816b\u760d\u306e\u767a\u751f\u306e\u5371\u967a\u7387\u306f\u653e\u5c04\u7dda\u6cbb\u7642\u3092\u53d7\u3051\u308b\u3068\uff13\u500d\u306b\u306a\u308b\u3068\u3044\u3046\u3053\u3068\u3067\u3059\u3002NF-1\u306e\u5b50\u4f9b\u306b\u306f\u8133\u816b\u760d\u306b\u5bfe\u3059\u308b\u653e\u5c04\u7dda\u6cbb\u7642\u306f\u3088\u307b\u3069\u306e\u7406\u7531\u304c\u306a\u3044\u3068\u884c\u308f\u306a\u3044\u3068\u7d50\u8ad6\u3055\u308c\u3066\u3044\u307e\u3059\u3002<\/p>\n<h4>NF-1\u306e\u57fa\u790e\u77e5\u8b58<\/h4>\n<p style=\"text-align: left;\"><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1cafe.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-2057\" title=\"nfforehead.jpg\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1cafe.jpg\" alt=\"\" width=\"600\" height=\"162\" \/><\/a>\u984d\u306b\u3067\u304d\u305f\u76ae\u819a\u306e\u591a\u767a\u6027\u7dda\u7dad\u816b neurofibroma \uff08\u304a\u3067\u3053\u306e\u30d6\u30c4\u30d6\u30c4\uff09\u3068\u8179\u90e8\u306e\u30ab\u30d5\u30a7\u30aa\u30ec\u6591 cafe-au-lait spot\uff08\u8336\u8272\u306e\u30b7\u30df\uff09\u3067\u3059\u3002\u3044\u305a\u308c\u3082\u826f\u6027\u306e\u75c5\u5909\u3067\u3059\u3002<\/p>\n<p style=\"text-align: left;\"><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1plexi.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-2058\" title=\"nfple.jpg\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/nf1plexi.jpg\" alt=\"\" width=\"600\" height=\"316\" \/><\/a>\u4e0b\u984e\u90e8\u304b\u3089\u54bd\u982d\u306b\u3067\u304d\u305f\u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b <span style=\"color: #000080;\"><strong>plexiform neurofibroma<\/strong><\/span> \u3067\u3059\u3002\u5916\u304b\u3089\u898b\u308b\u3068\u5927\u304d\u306a\u584a\u306e\u3088\u3046\u306b\u898b\u3048\u308b\u306e\u3067\u3059\u304c\uff0c\u7121\u6570\u306e\u5c0f\u3055\u306a\u795e\u7d4c\u306e\u816b\u760d\u304c\u96c6\u307e\u3063\u3066\u3067\u304d\u305f\u3082\u306e\u3067\u3059\u3002\u4e00\u3064\u4e00\u3064\u306f\u795e\u7d4c\u306b\u6cbf\u3063\u3066\u8513\u306e\u3088\u3046\u306b\u9577\u304f\u4f38\u3073\u3066\u3044\u308b\u306e\u3067\u8513\u72b6\u3068\u8a00\u3044\u307e\u3059\u3002<\/p>\n<ul>\n<li>\u4ed6\u306b\uff0c\u7b4b\u9aa8\u683c\u306e\u7570\u5e38\uff0c\u7cbe\u795e\u884c\u52d5\u306e\u8efd\u5ea6\u306e\u969c\u5bb3\uff0c\u8679\u5f69\u306e\u30ea\u30c3\u30b7\u30e5\u7d50\u7bc0 Lisch nodules\u306a\u3069\u304c\u307f\u3089\u308c\u307e\u3059<\/li>\n<li>\u65e5\u5e38\u751f\u6d3b\u306b\u652f\u969c\u3092\u304d\u305f\u3059\u3088\u3046\u306a\u75c7\u72b6\u3092\u5448\u3059\u308b\u306e\u306f20%\u307b\u3069\u306e\u60a3\u8005\u3055\u3093\u3067\uff0cNF-1\u306e8\u5272\u306e\u60a3\u8005\u3055\u3093\u306f\u3054\u304f\u666e\u901a\u306b\u751f\u6d3b\u3067\u304d\u307e\u3059<\/li>\n<li>NF-1\u3067\u6b7b\u4ea1\u3059\u308b\u3068\u3044\u3046\u3053\u3068\u306f\u307b\u3068\u3093\u3069\u3042\u308a\u307e\u305b\u3093<\/li>\n<li>\u307e\u308c\u3067\u3059\u304cNF-1\u95a2\u9023\u6b7b\u4ea1\u306e60%\u306fMPNST\u306e\u767a\u751f\u3068\u3055\u308c\u3066\u3044\u307e\u3059<\/li>\n<\/ul>\n<h5><span style=\"color: #003366;\">\u00a0\u795e\u7d4c\u7dda\u7dad\u816b\u75c7\u306f\uff0c\u77e5\u8b58\u306e\u6df1\u3044\u533b\u5e2b\u3068\u76f8\u8ac7\u3057\u306a\u304c\u3089\uff0c\u304d\u3061\u3093\u3068\u7ba1\u7406\u3059\u308c\u3070\uff0c\u5143\u6c17\u306b\u50cd\u3044\u3066\u751f\u304d\u3066\u3044\u304b\u308c\u308b\u75c5\u6c17\u3067\u3059\uff0c\u3042\u304d\u3089\u3081\u306a\u3044<\/span><\/h5>\n<p style=\"text-align: center;\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter wp-image-2084\" title=\"owlnf\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/owlnf.jpg\" alt=\"\" width=\"400\" height=\"577\" \/><\/p>\n<h5>\u304a\u307e\u3051\u306e\u77e5\u8b58<\/h5>\n<h5>\u907a\u4f1d\u5b50\u7570\u5e38<\/h5>\n<ul>\n<li>\u5e38\u67d3\u8272\u4f53\u512a\u6027\u907a\u4f1d\u3067\u3059<\/li>\n<li>\u7b2c17\u67d3\u8272\u4f53 (17q11.2) \u306e<span style=\"color: #000080;\"><strong>neurofibromin\u90e8\u5206\u6b20\u5931<\/strong><\/span>\uff08<span style=\"color: #000080;\"><strong><em>NF1<\/em>\u816b\u760d\u6291\u5236\u907a\u4f1d\u5b50\u5909\u7570<\/strong><\/span>\uff09\u306b\u3088\u3063\u3066\u751f\u3058\u307e\u3059<\/li>\n<li>neurofibromin\u306f\uff0cRas oncogene signal transduction pathway\u306enegative regulator\u3067\u3059<\/li>\n<li>\u5f93\u3063\u3066\uff0cRas\u304c\u5897\u5e45\u3055\u308c\u305f\u3088\u3046\u306a\u72b6\u614b\u3068\u306a\u308a\u816b\u760d\u5316\u3057\u307e\u3059<\/li>\n<li>plexiform neurofibroma\u306f\uff0celevated RAS-mitogen-activated protein kinase (MAPK) signaling\u304c\u7279\u5fb4\u3067\u3059<\/li>\n<li>\u3067\u3059\u304b\u3089MAPK kinase (MEK) 1 and 2\u963b\u5bb3\u5264\u304c\u6cbb\u7642\u85ac\u3068\u3057\u3066\u671f\u5f85\u3055\u308c\u3066\u3044\u307e\u3059<\/li>\n<\/ul>\n<hr \/>\n<\/div>\n<h4>plexiform neurofibroma \u53e2\u72b6\u795e\u7d4c\u7dda\u7dad\u816b\u306b selumetinib \u30bb\u30eb\u30e1\u30c1\u30cb\u30d6\u304c\u6709\u52b9<\/h4>\n<p><span class=\"title_default\">Andrea M: Selumetinib in Children with Inoperable Plexiform Neurofibromas. New Eng J Med, 2020<br \/>\n<span style=\"color: #000080;\"><strong>MEK 1\u963b\u5bb3\u5264 selumetinib<\/strong><\/span>\u306e\u81e8\u5e8a\u7b2c\uff12\u76f8\u8a66\u9a13\u306e\u7d50\u679c\u3067\u3059\u30022015\u5e74\u304b\u308950\u4eba\u306e\u60a3\u5150\u304c\u767b\u9332\u3055\u308c\u307e\u3057\u305f\u30021\u65e5\uff12\u56deselmetinib\u3092\u98f2\u307f\u307e\u3059\u30022019\u5e74\u306e\u6642\u70b9\u3067\uff0c35\u4eba (70%)\u3067partial response\u304c\u307f\u3089\u308c\uff0c\u305d\u306e\u3046\u306128\u4eba\u3067\u306f1\u5e74\u4ee5\u4e0a\u306e\u671f\u9593\u7dad\u6301\u3055\u308c\u307e\u3057\u305f\u3002\u60a3\u5150\u81ea\u8eab\u304c\u5831\u544a\u3057\u305f\u75db\u307f\u306e\u8efd\u6e1b\u306f2\/10\u3067\u81e8\u5e8a\u7684\u306b\u306f\u6539\u5584\u3068\u8a00\u3048\u307e\u3059\u3002PROs\uff08\u60a3\u8005\u5831\u544a\u30a2\u30a6\u30c8\u30ab\u30e0\uff09\u3067\uff0cQOL\uff0c\u7b4b\u529b\u3084\u53ef\u52d5\u57df\u306e\u6a5f\u80fd\u6539\u5584\u304c\u534a\u6570\u306e\u60a3\u8005\u3055\u3093\u3067\u5f97\u3089\u308c\u3066\u3044\u307e\u3059\u3002<\/span><span class=\"title_default\">\u624b\u8853\u3067\u304d\u306a\u3044\u8513\u72b6\u795e\u7d4c\u9798\u816b\u3092\u6709\u3059\u308b\u5b50\u4f9b\u305f\u3061\u306e\u591a\u304f\u3067\uff0c\u816b\u760d\u306e\u7e2e\u5c0f\u304c\u5f97\u3089\u308c\uff0c\u81e8\u5e8a\u52b9\u679c\u304c\u8a8d\u3081\u3089\u308c\u307e\u3057\u305f\u30025\u4f8b\u3067\u6709\u5bb3\u4e8b\u8c61\u306e\u305f\u3081\u306e\u4e2d\u65ad\uff0c6\u4f8b\u3067\u816b\u760d\u9032\u884c\u306e\u305f\u3081\u306e\u4e2d\u65ad\u304c\u3042\u308a\u307e\u3057\u305f\u3002\u6709\u5bb3\u4e8b\u8c61\u306f\uff0c\u5614\u6c17\uff0c\u5614\u5410\uff0c\u4e0b\u75e2\uff0c Cr\u4e0a\u6607\uff0c\u76ae\u75b9\u306a\u3069\u3067\u3059\u3002<\/span><\/p>\n<div class=\"setumei\">\n<h4>\u795e\u7d4c\u7dda\u7dad\u816b\u75c7\uff11\u578b\u306b\u767a\u751f\u3059\u308b\u30b0\u30ea\u30aa\u30fc\u30de\u306e\u6cbb\u7642<\/h4>\n<\/div>\n<p>Packer RJ: Implications of new understandings of gliomas in children and adults with NF1: report of a consensus conference.\u00a0Neuro-Oncology 2020<br \/>\n\u6982\u8ad6\u3067\u3059\uff1a\u307b\u3068\u3093\u3069\u304cindolent disease\u306a\u306e\u3067\u6cbb\u7642\u306e\u5fc5\u8981\u304c\u3042\u308a\u307e\u305b\u3093\u3002\u75c7\u5019\u6027\u3068\u306a\u308a\u6cbb\u7642\u304c\u5fc5\u8981\u306b\u306a\u308b\u3082\u306e\u306e\u4ee3\u8868\u4f8b\u306f\u8996\u795e\u7d4c\u81a0\u816b\u3067\uff0cNF-1\u306e15-20%\u306b\u767a\u751f\u3057\u307e\u3059\u3002\u8996\u795e\u7d4c\u81a0\u816b\u306e\u767a\u751f\u5e74\u9f62\u306e\u30d4\u30fc\u30af\u306f8\u6b73\u3067\uff0c18\u6b73\u307e\u3067\u304f\u3089\u3044\u3067\u53ef\u80fd\u6027\u304c\u3042\u308a\u307e\u3059\u3002NF-1\u6210\u4eba\u306e\u6bdb\u69d8\u7d30\u80de\u6027\u661f\u7d30\u80de\u816b\u3068\u4f4e\u60aa\u6027\u5ea6\u30b0\u30ea\u30aa\u30fc\u30de\u306f\uff0c\u975eNF-1\u3088\u308a\u4e88\u5f8c\u304c\u60aa\u3044\u3068\u3055\u308c\u307e\u3059\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u306f\u816b\u760d\u306e\u60aa\u6027\u5316\u3092\u8a98\u767a\u3059\u308b\u306e\u3067\u306a\u308b\u3079\u304f\u884c\u308f\u306a\u3044\u3002\u5e74\u9577\u5150\u3068\u6210\u4eba\u306b\u767a\u751f\u3059\u308b anaplastic piloid astrocytoma\u306b\u5bfe\u3059\u308b\u6cbb\u7642\u6cd5\u306f\u4e0d\u660e\u3067\u4e88\u5f8c\u304c\u60aa\u3044\u306a\u3069\u306e\u8a18\u8ff0\u304c\u3042\u308a\u307e\u3059\u3002<\/p>\n<h4>NF-1\u306e\u5b50\u4f9b\u306e\u9032\u884c\u6027\u4f4e\u60aa\u6027\u5ea6\u30b0\u30ea\u30aa\u30fc\u30de\u306b\u5bfe\u3059\u308b\u30ab\u30eb\u30dc\u30d7\u30e9\u30c1\u30f3\u3068\u30d3\u30f3\u30af\u30ea\u30b9\u30c1\u30f3\u5316\u5b66\u7642\u6cd5<\/h4>\n<p class=\"cit\">Ater JL: Nonrandomized comparison of neurofibromatosis type 1 and non-neurofibromatosis type 1 children who received carboplatin and vincristine for progressive low-grade glioma: A report from the Children&#8217;s Oncology Group.\u00a0<span role=\"menubar\">Cancer.<\/span> 2016<br \/>\n\u30ab\u30eb\u30dc\u30d7\u30e9\u30c1\u30f3\u3068\u30d3\u30f3\u30af\u30ea\u30b9\u30c1\u30f3\u4f75\u7528\u5316\u5b66\u7642\u6cd5\u306f\uff0cNF-1\u306e\u5b50\u4f9b\u306e\u4f4e\u60aa\u6027\u5ea6\u30b0\u30ea\u30aa\u30fc\u30de low-grade glioma\u306b\u6709\u52b9\u6027\u304c\u9ad8\u3044\u3068\u306e\u5831\u544a\u3067\u3059\u3002NF-1\u306b\u5bfe\u3057\u3066\u306e5\u5e74\u7121\u5897\u60aa\u751f\u5b58\u5272\u5408\u306f69%\u3067\u3042\u3063\u305f\u306e\u306b\u5bfe\u3057\u3066\uff0cNF-1\u3067\u306a\u3044\u5b50\u4f9b\u3067\u306f39%\u3067\u3057\u305f\u3002\u5bfe\u8c61\u3068\u3055\u308c\u305f\u75c7\u4f8b\u306e\u591a\u304f\u306f\u6bdb\u69d8\u7d30\u80de\u6027\u661f\u7d30\u80de\u816b\u3067\u3042\u3063\u305f\u3053\u3068\u306b\u6ce8\u610f\u3067\u3059\u3002<\/p>\n<p><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/owlnf.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-2084\" title=\"owlnf\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2012\/04\/owlnf.jpg\" alt=\"\" width=\"600\" height=\"865\" \/><\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>NF-1 neurofibromatosis type 1 \u89aa\u304b\u3089\u5b50\u306b\u907a\u4f1d\uff08\u5e38\u67d3\u8272\u4f53\u512a\u6027\u907a\u4f1d\u75be\u60a3\uff09\u3059\u308b\u3053\u3068\u3082\u3042\u308a\u307e\u3059\u304c\uff0c\u534a\u5206\u4ee5\u4e0a\u306e\u60a3\u8005\u3055\u3093\u306f\u907a\u4f1d\u3067\u306f\u3042\u308a\u307e\u305b\u3093 \uff11\u578b (NF-1 \u30a8\u30cc\u30a8\u30d5\u30ef\u30f3)\u00a0\u306f\uff0c\u5168\u8eab\u306e\u76ae\u819a\u306b\u305f\u304f\u3055\u3093\u306e [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[4],"tags":[],"class_list":["post-8100","post","type-post","status-publish","format-standard","hentry","category-braintumors"],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8100","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\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/comments?post=8100"}],"version-history":[{"count":28,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8100\/revisions"}],"predecessor-version":[{"id":8103,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8100\/revisions\/8103"}],"wp:attachment":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/media?parent=8100"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/categories?post=8100"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/tags?post=8100"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}