{"id":8914,"date":"2016-05-23T07:08:13","date_gmt":"2016-05-22T22:08:13","guid":{"rendered":"\/sawamura\/?p=8914"},"modified":"2025-09-09T18:34:31","modified_gmt":"2025-09-09T09:34:31","slug":"diffusemidlineglioma","status":"publish","type":"post","link":"https:\/\/plaza.umin.ac.jp\/sawamura\/glioma\/diffusemidlineglioma\/","title":{"rendered":"\u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de diffuse midline glioma"},"content":{"rendered":"<ul>\n<li>MRI\u3067\uff0c<strong><span style=\"color: #000080;\">\u982d\u306e\u771f\u3093\u4e2d\u306b\u5883\u754c\u304c\u306f\u3063\u304d\u308a\u3057\u306a\u3044\u306b\u3058\u3080\u3088\u3046\u306a\u816b\u760d<\/span><\/strong>\u304c\u307f\u3048\u305f\u3068\u304d\u306b\u7591\u3044\u307e\u3059<\/li>\n<li>WHO\u306e\u5206\u985e\u3067\u306f\uff0cdiffuse midline glioma, H3 K27M-mutant\u3068\u3044\u3044\u307e\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>histone H3-K27M<\/strong><\/span>\u3068\u3044\u3046\u907a\u4f1d\u5b50\u306b\u5909\u7570\u304c\u3042\u308b\u30b0\u30ea\u30aa\u30fc\u30de\u3067\u3059<\/li>\n<li>\u5c0f\u5150\u3068\u82e5\u5e74\u6210\u4eba\u306e\u4e2d\u67a2\u795e\u7d4c\u306e\u771f\u3093\u4e2d\u306b\u767a\u751f\u3059\u308b\u306e\u3067\u3053\u306e\u547c\u3073\u540d\u304c\u3064\u304d\u307e\u3057\u305f<\/li>\n<li><strong><span style=\"color: #ff0000;\">\u8996\u5e8a\u4e0b\u90e8\uff0c\u8996\u5e8a\uff0c\u6a4b\uff0c\u810a\u9ac4<\/span><\/strong>\u306b\u767a\u751f\u3057\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong><a style=\"color: #000080;\" href=\"\/sawamura\/glioma\/brainstemglioma\/\">\u3073\u307e\u3093\u6027\u6a4b\u81a0\u816b\uff08\u6a4b\u30b0\u30ea\u30aa\u30fc\u30de\uff0cDIPG) <\/a><\/strong><\/span>\u304c\u307b\u3068\u3093\u3069\u3092\u5360\u3081\u307e\u3059\u306e\u3067\uff0c\u5225\u306e\u30da\u30fc\u30b8\u306b\u3042\u308b\u6a4b\u30b0\u30ea\u30aa\u30fc\u30de\u306e\u3068\u3053\u308d\u3092\u8aad\u3093\u3067\u304f\u3060\u3055\u3044<\/li>\n<li>\u6a4b\u30b0\u30ea\u30aa\u30fc\u30de\u3068\u540c\u3058\u81e8\u5e8a\u50cf\u3067\u3059\u304b\u3089\u6cbb\u7642\u65b9\u6cd5\u3082\u540c\u3058\u3067\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong>\u6a4b\u306b\u767a\u751f\u3059\u308b\u3082\u306e\u306e\u5e74\u9f62\u4e2d\u592e\u5024\u306f7\u6b73\u304f\u3089\u3044\uff0c\u8996\u5e8a\u3084\u810a\u9ac4\u306e\u3082\u306e\u306f20\u6b73\u3092\u8d85\u3048\u307e\u3059<\/strong><\/span><\/li>\n<li>\u3068\u3066\u3082\u60aa\u6027\u5ea6\u306e\u9ad8\u3044\u30b0\u30ea\u30aa\u30fc\u30de\u3067\uff0c\u4e88\u5f8c\u4e0d\u826f\u3067\u3059<\/li>\n<li>2025\u5e74\u306e\u5831\u544a\u3067\uff0c2\u5e74\u751f\u5b58\u5272\u5408\u306f10\uff05\u4ee5\u4e0b\u3067\u3059<\/li>\n<li>\u69d8\u3005\u306a\u75c5\u7406\u7d44\u7e54\u50cf\u3092\u793a\u3057\u3066HE\u67d3\u8272\u3067\u306f\u8a3a\u65ad\u304c\u3067\u304d\u307e\u305b\u3093<\/li>\n<\/ul>\n<h5><span style=\"color: #ff0000;\">2025\u5e748\u67086\u65e5\u7c73\u56fd\u3067 \u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de\u306b\u5bfe\u3057\u3066dordaviprone (ONC201) \u306e\u4f7f\u7528\u304c\u8a8d\u53ef\u3055\u308c\u307e\u3057\u305f<br \/>\n<span style=\"color: #000080;\">\u5546\u54c1\u540d \u30e2\u30c7\u30a4\u30bd\u00a0Modeyso<\/span><br \/>\n<\/span><br \/>\n1\u6b73\u4ee5\u4e0a\u3067H3K27\u5909\u7570\u304c\u3042\u308a\uff0c\u518d\u767a\u306e\u60a3\u8005\u3055\u3093\u306b\u5bfe\u3057\u3066\u306e\u307f\u3067\u3059\uff0c\u9031\u306b\u4e00\u56de\uff0c\uff11\u30ab\u30d7\u30bb\u30eb\u3092\u98f2\u307f\u307e\u3059<\/p>\n<p><span style=\"color: #ff0000;\">DIPG\u306e\u60a3\u8005\u3055\u3093\u306f\u9664\u5916\u3055\u308c\u3066\u3044\u307e\u3059<\/span><\/h5>\n<p><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-14739\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial-300x295.jpg\" alt=\"\" width=\"300\" height=\"295\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial-300x295.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial-550x541.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial-768x755.jpg 768w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/dipgtmaxial.jpg 850w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><br \/>\n\u5e7c\u5c0f\u5150\u306b\u767a\u751f\u3059\u308bDIPG \u3073\u307e\u3093\u6027\u6a4b\u30b0\u30ea\u30aa\u30fc\u30de\u3067\u3059\u3002\u307b\u3068\u3093\u3069\u306e\u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de\u306fDIPG\u3067\u3059\u3002\u8133\u5e79\u90e8\u306e\u6a4b\u306e\u4e2d\u592e\u306b\u767a\u751f\u3057\uff0c\u6a4b\u304c\u816b\u5927\u3057\u307e\u3059\u3002<\/p>\n<p><a href=\"\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-9081\" src=\"\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass-300x298.jpg\" alt=\"midlinegliomass\" width=\"300\" height=\"298\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass-300x298.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass-200x200.jpg 200w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass-550x546.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass-144x144.jpg 144w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2016\/05\/midlinegliomass.jpg 567w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><br \/>\n20\u4ee3\u306e\u82e5\u3044\u60a3\u8005\u3055\u3093\u306e\u5ef6\u9ac4\u4e0b\u7aef\u306ediffuse midline glioma\u3067\u3059\uff0c\u7d44\u7e54\u8a3a\u65ad\u3067\u306fanaplastic astrocytoma\u3067\u3057\u305f<\/p>\n<h5>\u75c5\u7406\u50cf\u306f\uff1f\u3000\u96d1\u591a\u3067\u306a\u3093\u3067\u3082\u3042\u308a<\/h5>\n<p>\u4ee5\u4e0b\u306e\u3088\u3046\u306a\u90e8\u5206\u50cf\u3092\u793a\u3057\u307e\u3059<br \/>\ngliomas with giant cells, epithelioid and rhabdoid cells, primitive neuroectodermal tumor (PNET)-like foci, neuropil-like islands, pilomyxoid features, ependymal-like areas, sarcomatous transformation, ganglionic differentiation, and pleomorphic xanthoastrocytoma (PXA)-like area<\/p>\n<h5>\u30b0\u30ea\u30aa\u30fc\u30de\u306a\u3089\u306a\u3093\u3067\u3082\u6df7\u3058\u3063\u3066\u3088\u3044\u3068\u3044\u3046\u3053\u3068\u3067\u3059\u304b\u3089\uff0c\u907a\u4f1d\u5b50\u8a3a\u65ad\u3057\u306a\u3044\u3068\u308f\u304b\u308a\u307e\u305b\u3093<\/h5>\n<p>IDH1 mutation\u3068EGFR amplification\u306f\u3042\u308a\u307e\u305b\u3093\u304c\uff0c\u307e\u308c\u306bco-occurred with BRAF-V600E mutation\u304c\u3042\u308a\u307e\u3059\u3002p53 over expression\u3068ATRX loss (except in pontine gliomas)\u3068monosomy 10\u304c\u8a8d\u3081\u3089\u308c\u308b\u3053\u3068\u306f\u591a\u3044\u3067\u3059\u3002<\/p>\n<h4>\u6cbb\u7642\u306f\u3069\u3046\u3059\u308b\u306e\u304b<\/h4>\n<ul>\n<li>\u5916\u79d1\u6cbb\u7642\uff08\u958b\u982d\u816b\u760d\u6458\u51fa\u8853\uff09\u306e\u6709\u52b9\u6027\u306f\u307b\u3068\u3093\u3069\u3042\u308a\u307e\u305b\u3093<\/li>\n<li>\u6700\u3082\u591a\u3044\u3073\u307e\u3093\u6027\u6a4b\u81a0\u816b\u3067\u306f\u751f\u691c\u8853\u306f\u3057\u307e\u305b\u3093<\/li>\n<li>\u8996\u5e8a\u306e\u816b\u760d\u306f\u751f\u691c\u624b\u8853\uff08\u75c5\u7406\u8a3a\u65ad\uff09\u304c\u306a\u3055\u308c\u307e\u3059<\/li>\n<li>\u5c40\u6240\u653e\u5c04\u7dda\u6cbb\u7642\u304c\u4e16\u754c\u6a19\u6e96\u7684\u306a\u6cbb\u7642\u3067\u3059<\/li>\n<li>\u751f\u691c\u8853\u3067\u75c5\u7406\u7d44\u7e54\u8a3a\u65ad\u304c\u3067\u308c\u3070\uff0c\u907a\u4f1d\u5b50\u8a3a\u65ad\u3067diffuse midline glioma\u3067\u3042\u3063\u3066\u3082\uff0c<span style=\"color: #000080;\"><strong>WHO\u30b0\u30ec\u30fc\u30c9\u3068\u7d44\u7e54\u50cf\u306b\u5fdc\u3058\u305f\u6cbb\u7642\u304c<\/strong><\/span>\u306a\u3055\u308c\u307e\u3059<\/li>\n<li>\u5206\u5b50\u6a19\u7684\u6cbb\u7642\u85ac\u3068\u3057\u3066OCN201\u304c\u671f\u5f85\u3055\u308c\u3066\u6cbb\u9a13\u4e2d\u3067\u3059\uff0c\u6709\u52b9\u6027\u306f\u5fc5\u305a\u3057\u3082\u671f\u5f85\u307b\u3069\u3067\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<li>2025\u5e741\u6708\u306e\u6bb5\u968e\u3067\u306f\uff0c\u653e\u5c04\u7dda\u306b\u6b21\u3050\u78ba\u305f\u308b\u6cbb\u7642\u65b9\u6cd5\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<\/ul>\n<div class=\"setumei\">\n<h5><a href=\"https:\/\/www.ncc.go.jp\/jp\/ncch\/division\/clinical_trial\/patient\/to_patients\/03\/T5272.html\">\u56fd\u7acb\u304c\u3093\u30bb\u30f3\u30bf\u30fc\u306eONC201\u81e8\u5e8a\u8a66\u9a13<\/a><br \/>\nhttps:\/\/www.ncc.go.jp\/jp\/ncch\/division\/clinical_trial\/patient\/to_patients\/03\/T5272.html<\/h5>\n<\/div>\n<ul>\n<li>2025\u5e744\u6708\u306b\u65e5\u672c\u3067\u3082\u958b\u59cb\u3055\u308c\u307e\u3057\u305f<\/li>\n<li>\u6295\u4e0e\u85ac\u5264\u306f\uff0c\u90311\u56de\u3042\u308b\u3044\u306f2\u56de\u306eONC201\u3067\u3059<\/li>\n<li>\u5bfe\u8c61\u306f\uff0cH3K27M\u5909\u7570\u304c\u3042\u308b\u3068\u8a3c\u660e\u3055\u308c\u305f\u3073\u307e\u3093\u6027\u795e\u7d4c\u81a0\u816b\u3067\u3059<\/li>\n<li>\u4f53\u91cd\u304c10kg\u4ee5\u4e0a\uff0c\u653e\u5c04\u7dda\u5024\u9577\u3092\u3059\u3067\u306b\u53d7\u3051\u3066\u3044\u308b\u3068\u3044\u3046\u3088\u3046\u306a\u6761\u4ef6\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>ONC201\u304c\u65e5\u672c\u3067\u4f7f\u7528\u3067\u304d\u308b\u552f\u4e00\u306e\u7a93\u53e3\u3067\u3059<\/li>\n<\/ul>\n<hr \/>\n<h1><span style=\"color: #000080;\"><a style=\"color: #000080;\" href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/glioma\/brainstemglioma\/\">\u6a4b\u30b0\u30ea\u30aa\u30fc\u30de\uff0c\u3073\u307e\u3093\u6027\u6a4b\u81a0\u816b DIPG\u306f\u3053\u3053\u3092\u30af\u30ea\u30c3\u30af<\/a><\/span><\/h1>\n<h5>\u6587\u732e<\/h5>\n<h4>ONC201\u306f\uff0c\u518d\u767a\u3057\u305f\u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de\u306b\u308f\u305a\u304b\u306a\u304c\u3089\u52b9\u679c\u304c\u8a8d\u3081\u3089\u308c\u308b<\/h4>\n<p class=\"heading-title\"><span class=\"authors-list-item \">Arrillaga-Romany I:\u00a0<\/span>ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma. J Clin Oncol 2024<br \/>\n50\u4f8b\u306e\u518d\u767a\u60a3\u8005\u3055\u3093\u3067ONC201\u306e\u52b9\u679c\u304c\u8a66\u3055\u308c\u307e\u3057\u305f\u3002\u6210\u4eba\u304c46\u540d\uff0c18\u6b73\u4ee5\u4e0b\u306f4\u540d\u3067\u3059\u3002\u653e\u5c04\u7dda\u6cbb\u7642\u304b\u3089\u306f90\u65e5\u4ee5\u4e0a\u304c\u7d4c\u904e\u3057\u3066\u304a\u308a\uff0cPS\u306f60\u4ee5\u4e0a\u3067\uff0c\u3073\u307e\u3093\u6027\u6a4b\u81a0\u816b DIPG \u306f\u9664\u5916\u3055\u308c\u3066\u3044\u307e\u3059\u3002ORR\uff08\u5168\u594f\u529f\u5272\u5408 RANO-HGG\uff09\u306f20\uff05\u3067\u3057\u305f\u3002TTR\u4e2d\u592e\u5024\u306f8.3\u30f6\u6708\uff0cDOR\uff08\u6709\u52b9\u671f\u9593\uff09\u4e2d\u592e\u5024\u306f11.2 \u30f6\u6708\u3067\u3057\u305f\u3002PS\u306e\u6539\u5584\uff08\u75c7\u72b6\u306e\u6539\u5584\uff09\u306f\u8a55\u4fa1\u3057\u3046\u308b34\u4eba\u306e\u51856\u4eba\u306b\u8a8d\u3081\u3089\u308c\u307e\u3057\u305f\u3002<\/p>\n<h4>BRAF\/FGFR1\u5909\u7570\u3092\u4f34\u3046\u30b5\u30d6\u30bf\u30a4\u30d7\u306f\u4e88\u5f8c\u304c\u5c11\u3057\u826f\u3044\u304b\u3082<\/h4>\n<p class=\"heading-title\">Auffret L: A new subtype of diffuse midline glioma, H3 K27 and BRAF\/FGFR1 co-altered: a clinico-radiological and histomolecular characterisation. Acta Neuropathol 2023<br \/>\nH3-K27\u306b\u5909\u7570\u304c\u3042\u308b\u3073\u307e\u3093\u6027\u6b63\u4e2d\u30b0\u30ea\u30aa\u30fc\u30de\u306e\u4e2d\u306b\uff0cBRAF\u3082\u3057\u304f\u306fFGFR1\u306e\u5909\u7570\u3092\u4f34\u3046\u3082\u306e\u304c\u3042\u308a\u307e\u3059\u300260\u4f8b\u3067\u81e8\u5e8a\u50cf\u304c\u89e3\u6790\u3055\u308c\u307e\u3057\u305f\u3002\u8996\u5e8a\u306b\u767a\u751f\u983b\u5ea6\u304c\u9ad8\u304f(70% for BRAF<sup>V600E<\/sup> and 58% for FGFR1<sup>MUT<\/sup>) \u77f3\u7070\u5316\u304c\u8a8d\u3081\u3089\u308c\u305f\u3068\u306e\u3053\u3068\u3067\u3059\u3002OS\u304c\u6bd4\u8f03\u7684\u9577\u304f\u4e2d\u592e\u5024\u30673\u5e74\u3092\u8d8a\u3048\u308b\u3068\u306e\u3053\u3068\u3067\u3059\u3002<\/p>\n<h4>\u62e1\u6563\u5f37\u8abf\u753b\u50cf\u3067\u306e\u9ad8\u4fe1\u53f7\u3068\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u5897\u5f37\u304c\u4e00\u81f4\u3057\u306a\u3044\u3082\u306e\u306f\u4e88\u5f8c\u304c\u60aa\u3044\u304b\u3082\u3057\u308c\u306a\u3044 DWI-Gd mismach<\/h4>\n<p class=\"heading-title\">Ikeda K: Diffusion-weighted imaging-gadolinium enhancement mismatch sign in diffuse midline glioma. Eur J Radiol 2022<br \/>\nDWI-Gd mismatch sign\u304c\u3042\u308b\u3082\u306e\u306e\u4e88\u5f8c\u304c\u60aa\u3044\u304b\u3082\u3057\u308c\u306a\u3044\u3068\u3044\u3046\u3053\u3068\u3067\u3059\u3002\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u5897\u5f37\u3055\u308c\u308b\u90e8\u4f4d\u306e\u62e1\u6563\u5f37\u8abf\u753b\u50cf\u304c\u5f37\u3044\u9ad8\u4fe1\u53f7\u3068\u306a\u308b\u3082\u306e\u306e\u4e88\u5f8c\u304c\u6700\u3082\u77ed\u3044\u3068\u3044\u3046\u5831\u544a\u3067\u3057\u305f\u3002<\/p>\n<h4>\u6587\u732e<\/h4>\n<p>Solomon DA, et al.: Diffuse Midline Gliomas with Histone H3-K27M Mutation: A Series of 47 Cases Assessing the Spectrum of Morphologic Variation and Associated Genetic Alterations. Brain Pathol 10, 2015<\/p>\n","protected":false},"excerpt":{"rendered":"<p>MRI\u3067\uff0c\u982d\u306e\u771f\u3093\u4e2d\u306b\u5883\u754c\u304c\u306f\u3063\u304d\u308a\u3057\u306a\u3044\u306b\u3058\u3080\u3088\u3046\u306a\u816b\u760d\u304c\u307f\u3048\u305f\u3068\u304d\u306b\u7591\u3044\u307e\u3059 WHO\u306e\u5206\u985e\u3067\u306f\uff0cdiffuse midline glioma, H3 K27M-mutant\u3068\u3044\u3044\u307e\u3059 histone H3-K27M\u3068 [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[16],"tags":[],"class_list":["post-8914","post","type-post","status-publish","format-standard","hentry","category-glioma"],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8914","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=8914"}],"version-history":[{"count":27,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8914\/revisions"}],"predecessor-version":[{"id":23661,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/posts\/8914\/revisions\/23661"}],"wp:attachment":[{"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/media?parent=8914"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/categories?post=8914"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/plaza.umin.ac.jp\/sawamura\/wp-json\/wp\/v2\/tags?post=8914"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}