{"id":4006,"date":"2013-09-15T06:30:27","date_gmt":"2013-09-14T21:30:27","guid":{"rendered":"\/sawamura\/?p=4006"},"modified":"2022-06-25T10:13:33","modified_gmt":"2022-06-25T01:13:33","slug":"lhermitteduclosdisease","status":"publish","type":"post","link":"https:\/\/plaza.umin.ac.jp\/sawamura\/pediattumor\/lhermitteduclosdisease\/","title":{"rendered":"\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5  Lhermitte-Duclos disease"},"content":{"rendered":"<h2><span style=\"color: #000080;\"><span style=\"color: #ff0000;\">\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5<\/span> \u00a0Lhermitte-Duclos disease<\/span><br \/>\n<span style=\"color: #000080;\">\u5c0f\u8133\u7570\u5f62\u6210\u6027\u795e\u7d4c\u7bc0\u7d30\u80de\u816b \u00a0Dysplastic gangliocytoma of the cerebellum\u00a0<\/span><\/h2>\n<ul>\n<li>\u4e73\u5150\u304b\u308960\u4ee3\u307e\u3067\uff0c\u5e45\u5e83\u3044\u5e74\u9f62\u3067\u767a\u75c7\u3057\u307e\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>20\u4ee3\u304b\u308930\u4ee3\u306b\u591a\u3044<\/strong><\/span>\u3067\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>WHO grade 1\u306e\u826f\u6027\u816b\u760d<\/strong><span style=\"color: #000000;\">\u3067\u3059<\/span><\/span><\/li>\n<li>\u5de6\u53f3\u3069\u3061\u3089\u304b\u306e<span style=\"color: #ff0000;\"><strong>\u5c0f\u8133\u534a\u7403\u304c\u816b\u308c\u3066\u5927\u304d\u304f\u306a\u3063\u3066\u3057\u307e\u3046<\/strong><\/span>\u75c5\u6c17\u3067\u3059<\/li>\n<li>\u816b\u760d tumor\/neoplam \u3068\u3044\u3046\u3088\u308a\u3082\uff0c\u5c0f\u8133\u306e\u4e00\u90e8\u304c\u7570\u5e38\u306b\u5927\u304d\u304f\u306a\u3063\u3066\u3057\u307e\u3046\u3082\u306e\u3068\u8003\u3048\u305f\u65b9\u304c\u3044\u3044\u3067\u3057\u3087\u3046<\/li>\n<li>\u904e\u8aa4\u816b hamartoma \u3042\u308b\u3044\u306f\u5f62\u6210\u7570\u5e38 dysplasia \u3068\u3082\u3044\u308f\u308c\u307e\u3059<\/li>\n<li>\u3067\u3082\uff0c\u3068\u3066\u3082\u3086\u3063\u304f\u308a\u4f55\u5e74\u3082\u304b\u3051\u3066\u5927\u304d\u304f\u306a\u308a\u307e\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>\u6c34\u982d\u75c7\u3092\u5408\u4f75\u3059\u308b<\/strong><\/span>\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li>\u5c0f\u8133\u304c\u816b\u308c\u308b\u306e\u3067\u5f8c\u982d\u7aa9\uff08\u30c6\u30f3\u30c8\u4e0b\uff09\u306e\u5727\u304c\u4e0a\u6607\u3057\u3066\u75c7\u72b6\u304c\u51fa\u307e\u3059<\/li>\n<\/ul>\n<h4>\u539f\u56e0<\/h4>\n<div>\n<ul>\n<li>\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4\u306b\u5408\u4f75\u3059\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>PTEN\u907a\u4f1d\u5b50\u306b\u7570\u5e38<\/strong><\/span>\u304c\u3042\u3063\u3066\u751f\u307e\u308c\u305f\u4eba\u306b\u751f\u3058\u307e\u3059<\/li>\n<\/ul>\n<\/div>\n<h4>\u75c7\u72b6<\/h4>\n<div>\n<ul>\n<li>\u6570\u5e74\u304b\u304b\u3063\u3066\u3086\u3063\u304f\u308a\u75c7\u72b6\u304c\u9032\u884c\u3059\u308b\u3053\u3068\u304c\u591a\u3044\u3067\u3059<\/li>\n<li>\u982d\u306e\u4e2d\u306e\u5727\u529b\u304c\u3042\u304c\u308b\u8133\u5727\u4ea2\u9032\u3068\u3044\u3046\u75c7\u72b6\u304c\u51fa\u307e\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>\u982d\u75db\uff0c\u5455\u5410\uff0c\u76ee\u304c\u307c\u3084\u3051\u308b<\/strong><\/span>\uff08\u8996\u529b\u969c\u5bb3\uff09\uff0c2\u91cd\u306b\u898b\u3048\u308b\uff08\u8907\u8996\uff09\u306a\u3069\u3067\u3059<\/li>\n<li>\u4e73\u5150\u3067\u306f\uff0c\u773c\u632f\u3084\u5455\u5410\u306a\u3069\u306e\u75c7\u72b6\u304c\u307f\u3089\u308c\u307e\u3059<\/li>\n<li>\u307e\u308c\u306b\uff0c\u6025\u6027\u306e\u9589\u585e\u6027\u6c34\u982d\u75c7\u3067\uff0c\u50be\u7720\u50be\u5411\u3084\u610f\u8b58\u969c\u5bb3\u306b\u306a\u308a\u307e\u3059<\/li>\n<li>\u6b69\u304f\u6642\u306b\u3075\u3089\u3064\u304f\uff0c\u4e0d\u5b89\u5b9a\u306b\u306a\u308b\u3068\u3044\u3046<span style=\"color: #ff0000;\"><strong>\u5c0f\u8133\u5931\u8abf\u3082\u591a\u3044<\/strong><\/span>\u3067\u3059<\/li>\n<li>\u8133\u795e\u7d4c\u9ebb\u75fa\u3082\u307f\u3089\u308c\uff0c\u7247\u65b9\u306e\u8074\u529b\u304c\u4f4e\u4e0b\u3059\u308b\uff0c\u98f2\u307f\u8fbc\u307f\u304c\u60aa\u3044\uff08\u56a5\u4e0b\u969c\u5bb3\uff09\u306a\u3069\u304c\u307f\u3089\u308c\u307e\u3059<\/li>\n<li>\u5927\u982d\u75c7\uff0c\u591a\u6307\u75c7\uff0c\u591a\u767a\u6027\u8840\u7ba1\u816b\uff0c\u982d\u84cb\u9aa8\u5947\u5f62\uff08\u65e9\u671f\u7652\u5408\u75c7\uff09\u306a\u3069\u3092\u5408\u4f75\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<\/ul>\n<h4>\u8a3a\u65ad<\/h4>\n<ul>\n<li><strong><span style=\"color: #ff0000;\">MRI\u3067\u78ba\u5b9a\u8a3a\u65ad\u304c\u3067\u304d\u307e\u3059<\/span><\/strong><\/li>\n<li>MRI\u3067\u7247\u65b9\u306e\u5c0f\u8133\u306b\u5927\u304d\u306a\u816b\u760d\u304c\u307f\u3089\u308c\u307e\u3059<\/li>\n<li>T1\u5f37\u8abf\u753b\u50cf\u3067\u4f4e\u4fe1\u53f7\uff0cT2\u5f37\u8abf\u753b\u50cf\u3067\u3084\u3084\u9ad8\u4fe1\u53f7\u306b\u306a\u308a\u307e\u3059<\/li>\n<li>\u30ac\u30c9\u30ea\u30cb\u30a6\u30e0\u3067\u5897\u5f37\u3055\u308c\u306a\u3044\u3053\u3068\u3082\u3042\u308a\u307e\u3059\u304c\uff0c\u4e00\u90e8\u304c\u5897\u5f37\u3055\u308c\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u304b\u3064\u3066\u306f\uff0ca cerebellum in the cerebellum\u3068\u3044\u308f\u308c\u305f\u3088\u3046\u306b\uff0c\u5c0f\u8133\u306e\u4e2d\u306b\u5225\u306a\u5c0f\u8133\u304c\u3067\u304d\u305f\u3088\u3046\u306a\u5f62\u614b\u3092\u793a\u3057\u307e\u3059<\/li>\n<li>gyriform pattern, laminated lesion, striated appearance, tiger-stripe appearance \u3068\u8868\u73fe\u3055\u308c\u308b\u6240\u898b\u3067\u8a3a\u65ad\u304c\u3064\u304d\u307e\u3059<\/li>\n<li>FDG-PET\u3067\u306f\u6b63\u5e38\u5c0f\u8133\u3088\u308a\u3082\u5f37\u3044uptake\u304c\u307f\u3089\u308c\u307e\u3059\u304c\u7de9\u5f90\u5897\u5927\u6027\u306e\u75c5\u5909\u3067\u3059<\/li>\n<\/ul>\n<h4>1\u6b73\u3067\u6c34\u982d\u75c7\u3068\u5de6\u8074\u529b\u4f4e\u4e0b\u3067\u767a\u75c7\u3057\u305f\u4f8b<\/h4>\n<\/div>\n<div><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet1-1.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-19799\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet1-1-292x300.jpg\" alt=\"\" width=\"292\" height=\"300\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet1-1-292x300.jpg 292w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet1-1-550x564.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet1-1.jpg 650w\" sizes=\"auto, (max-width: 292px) 100vw, 292px\" \/><\/a><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet2-1.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-19800\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet2-1-288x300.jpg\" alt=\"\" width=\"288\" height=\"300\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet2-1-288x300.jpg 288w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet2-1-550x572.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermittet2-1.jpg 677w\" sizes=\"auto, (max-width: 288px) 100vw, 288px\" \/><\/a><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div><\/div>\n<div>\u6c34\u982d\u75c7\u306b\u3088\u308b\u5614\u5410\u3068\u8efd\u5ea6\u306e\u610f\u8b58\u969c\u5bb3\u3067\u767a\u75c7\u3057\u307e\u3057\u305f\u3002\u6cbb\u7642\u306f\u5de6\u5c0f\u8133\u6a4b\u89d2\u90e8\u3067\u8133\u795e\u7d4c\u3092\u5727\u8feb\u3057\u3066\u3044\u308b\u90e8\u5206\u3060\u3051\u3092\u307b\u3093\u306e\u5c11\u3057\u90e8\u5206\u6458\u51fa\u3057\u307e\u3057\u305f\u3002\u6c34\u982d\u75c7\u306f\u5185\u8996\u93e1\u306b\u3088\u308b\u7b2c\uff13\u8133\u5ba4\u958b\u7a93\u8853\u3067\u6cbb\u3057\u307e\u3057\u305f\u3002<\/div>\n<div>\n<p><a href=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermitte.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-7643 size-medium\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermitte-300x288.jpg\" alt=\"lhermitte\" width=\"300\" height=\"288\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermitte-300x288.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermitte-550x528.jpg 550w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/lhermitte.jpg 850w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/a><br \/>\n\u4e0a\u306b\u304a\u898b\u305b\u3057\u305f\u4f8b\u306e\uff18\u5e74\u5f8c\u306e\u753b\u50cf\u3067\u3059\u3002\u816b\u760d\u306f8\u5e74\u9593\u3067\u5168\u304f\u5927\u304d\u304f\u306a\u308a\u307e\u305b\u3093\u3067\u3057\u305f\u3002<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft size-medium wp-image-19798\" src=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/rhermittemr-300x288.jpg\" alt=\"\" width=\"300\" height=\"288\" srcset=\"https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/rhermittemr-300x288.jpg 300w, https:\/\/plaza.umin.ac.jp\/sawamura\/wp\/wp-content\/uploads\/2013\/09\/rhermittemr.jpg 509w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><br \/>\n15\u5e74\u5f8c\uff0c17\u6b73\u306e\u6642\u306e\u753b\u50cf\u3067\u3059\u3002\u5168\u304f\u5909\u5316\u304c\u306a\u3044\u304b\u3054\u304f\u308f\u305a\u304b\u306b\u9000\u7e2e\u3057\u3066\u3044\u308b\u304b\u3082\u3057\u308c\u307e\u305b\u3093\u3002\u81ea\u8ee2\u8eca\u306b\u3082\u4e57\u308c\u307e\u3059\uff0c\u3061\u3087\u3063\u3068\u8d70\u308b\u3053\u3068\u304c\u9045\u3044\u666e\u901a\u306e\u9ad8\u6821\u751f\u3067\u3059\u3002<\/p>\n<\/div>\n<h4>\u6cbb\u7642<\/h4>\n<ul>\n<li><span style=\"color: #ff0000;\"><strong>\u5f8c\u982d\u4e0b\u958b\u982d\u3067\u816b\u760d\u5316\u3057\u305f\u90e8\u5206\u3060\u3051\u3092\u90e8\u5206\u6458\u51fa\u3057\u307e\u3059<\/strong><\/span><\/li>\n<li>\u5fc5\u305a\u3057\u3082\u816b\u760d\u3092\u5168\u90e8\u3068\u308b\u5fc5\u8981\u306f\u3042\u308a\u307e\u305b\u3093<\/li>\n<li>\u5927\u304d\u306a\u816b\u760d\u3092\u5168\u6458\u51fa\u3057\u3066\u3082\u75c7\u72b6\u3092\u6b8b\u3055\u306a\u3044\u3053\u3068\u3082\u591a\u3044\u3067\u3059<\/li>\n<li>\u6c34\u982d\u75c7\u3092\u5408\u4f75\u3057\u3066\u3044\u308b\u4f8b\u3067\u306f\uff0c<span style=\"color: #ff0000;\"><strong>\u5185\u8996\u93e1\u306b\u3088\u308b\u7b2c\uff13\u8133\u5ba4\u958b\u7a93\u8853\u3092\u3057\u307e\u3059<\/strong><\/span><\/li>\n<\/ul>\n<h4>\u75c5\u7406<\/h4>\n<div>\n<ul>\n<li>widening of the molecular layer<\/li>\n<li>proliferation of abnormal ganglion cells<\/li>\n<li>absence of the Purkinje cell layer<\/li>\n<li>hypertrophy of the granule cell layer<\/li>\n<\/ul>\n<\/div>\n<h4>\u4e88\u5f8c<\/h4>\n<ul>\n<li>\u826f\u6027\u306e\u7d4c\u904e\u3092\u305f\u3069\u308a\u307e\u3059<\/li>\n<li>\u52a0\u9f62\u3068\u3068\u3082\u306b\u601d\u6625\u671f\u3067\u81ea\u7136\u9000\u7e2e\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4\u3092\u5408\u4f75\u3057\u3066\u3044\u308b\u3068\uff0c\u764c\u3092\u767a\u75c7\u3059\u308b\u53ef\u80fd\u6027\u304c\u9ad8\u304f\u306a\u308a\u307e\u3059<\/li>\n<\/ul>\n<h4>\u60aa\u6027\u795e\u7d4c\u81a0\u816b\u3078\u9032\u5c55\u3059\u308b\u3053\u3068<\/h4>\n<div>\n<ul>\n<li>\u304d\u308f\u3081\u3066\u307e\u308c\u306b\uff0c\u904e\u8aa4\u816b\u6027\u795e\u7d4c\u7bc0\u7d30\u80de\u816b\u304b\u3089\uff0c\u795e\u7d4c\u7bc0\u81a0\u816b\u3092\u6df7\u3058\u3066\u30b0\u30ec\u30fc\u30c93\u306e\u795e\u7d4c\u7bc0\u81a0\u816b\u3078\u3068\u60aa\u6027\u5316\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>Takei H, et al.: Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. J Neurosurg 107: 137-142, 2007\u306b\u5c0f\u5150\u4f8b\u304c\u5831\u544a\u3055\u308c\u307e\u3057\u305f<\/li>\n<li>\u6fa4\u6751\u3082\u540c\u6642\u671f\u306b\u307b\u3068\u3093\u3069\u540c\u3058\u75c7\u4f8b\u3092\u7d4c\u9a13\u3057\u305f\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<\/ul>\n<\/div>\n<div>\n<hr \/>\n<\/div>\n<h3><span style=\"color: #000080;\">\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4 Cawden symdrome<\/span><\/h3>\n<ul>\n<li>multiple hamartoma-neoplasia syndrome\u306e\u4e00\u7a2e\u3067\u3059<\/li>\n<li>\u76ae\u819a\u79d1\u3067\u8a3a\u65ad\u3055\u308c\u308b\u3053\u3068\u306e\u591a\u3044\u75c5\u6c17\u3067\u3059<\/li>\n<li>\u5185\u8535\u3082\u542b\u3081\u3066\uff0c\u8eab\u4f53\u306e\u3042\u3061\u3053\u3061\u306b\u904e\u8aa4\u816b hamartoma\u3068\u3044\u3046\u826f\u6027\u306e\u3067\u304d\u3082\u306e\u304c\u3067\u304d\u307e\u3059<\/li>\n<li>\u7279\u306b<span style=\"color: #000080;\"><strong>\u9854\u9762\u3068\u53e3\u8154\u5185\u306b\uff0c\u591a\u767a\u6027\u306e\u4e73\u982d\u72b6\u4e18\u75b9 papule \u3084\u6bdb\u6839\u9798\u816b trichilemmoma\u304c\u3067\u304d\u307e\u3059<\/strong><\/span><\/li>\n<li>\u3053\u308c\u3089\u306e\u3067\u304d\u3082\u306e\u304c\u60aa\u6027\u5316\u3059\u308b\u3053\u3068\u3082\u3042\u308a\u307e\u3059<\/li>\n<li><span style=\"color: #000080;\"><strong>\u4e73\u304c\u3093\uff0c\u7532\u72b6\u817a\u304c\u3093\uff0c\u5b50\u5bae\u304c\u3093<\/strong><\/span>\u306a\u3069\u3067\u3059<\/li>\n<li><strong>\u307e\u308c\u306b\uff0c<\/strong><span style=\"color: #000080;\"><strong>\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5\u3092\u4f75\u767a\u3059\u308b<\/strong><\/span>\u3053\u3068\u3067\u3082\u77e5\u3089\u308c\u3066\u3044\u307e\u3059<\/li>\n<li>\u982d\u90e8\u3067\u306f\uff0c\u5927\u982d\u75c7 macrocephaly\u3092\u5408\u4f75\u3059\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059<\/li>\n<li>\u3067\u3059\u304b\u3089\uff0c\u5c0f\u8133\u816b\u760d\u304c\u3042\u3063\u3066\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5\u3068\u8a3a\u65ad\u3055\u308c\u305f\u5834\u5408\u306b\u306f\uff0c\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4\u3092\u4f75\u767a\u3057\u3066\u3044\u306a\u3044\u304b\u3069\u3046\u304b\u3092\u8abf\u3079\u307e\u3059<\/li>\n<\/ul>\n<h4>\u539f\u56e0<\/h4>\n<ul>\n<li>\u7b2c10\u67d3\u8272\u4f53\u9577\u8155\u306ePTEN\u907a\u4f1d\u5b50 (Phosphatase and Tensin homolog gene)\u306b\u5909\u7570\u304c\u3042\u308b\u305f\u3081\u306b\u751f\u3058\u308b\u75be\u60a3\u3067\u3059<\/li>\n<li>\u5e38\u67d3\u8272\u4f53\u512a\u6027\u907a\u4f1d\u3067\u3059<\/li>\n<\/ul>\n<hr \/>\n<h3><span style=\"color: #000080;\">PTEN hamartoma tumor syndrome (PHTS)<\/span><\/h3>\n<ul>\n<li>\u751f\u307e\u308c\u3064\u304dPTEN\u907a\u4f1d\u5b50\u5909\u7570 (germ line PTEN mutation) \u304c\u3042\u308b\u60a3\u8005\u3055\u3093\u306b\u751f\u3058\u308b\u3082\u306e\u3067\u3059<\/li>\n<li>\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4\uff0cBannayan-Riley-Ruvalcaba syndrome\uff0cProteus-like syndrome\u306a\u3069\u3092\u542b\u3081\u3066\uff0cPTEN hamartoma tumor syndrome\u3068\u3044\u3044\u307e\u3059<\/li>\n<li>PTEN\u306f\u7d30\u80de\u5468\u671f\u3092\u8abf\u7bc0\u3059\u308bphosphatase\u3092\u4ecb\u3057\u3066\u816b\u760d\u6291\u5236\u907a\u4f1d\u5b50\u3068\u3057\u3066\u50cd\u3044\u3066\u3044\u307e\u3059<\/li>\n<li>PTEN\u306e\u6a5f\u80fd\u304c\u5931\u308f\u308c\u308b\u3068\u7d30\u80de\u5897\u6b96\u304c\u30b3\u30f3\u30c8\u30ed\u30fc\u30eb\u3067\u304d\u306a\u304f\u306a\u308a\u816b\u760d\u304c\u767a\u751f\u3057\u307e\u3059<\/li>\n<li>\u826f\u6027\u306e\u904e\u8aa4\u816b\u304c\u3067\u304d\u308b\u3070\u304b\u308a\u3067\u306a\u304f\uff0c\u764c\u306e\u767a\u751f\u7387\u3082\u9ad8\u3044\u3067\u3059<\/li>\n<li><span style=\"color: #ff0000;\"><strong>\u30b3\u30fc\u30c7\u30f3\u75c7\u5019\u7fa4<\/strong><\/span>\u3084<strong><span style=\"color: #ff0000;\">\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5<\/span><\/strong>\u3092\u751f\u3058\u305f\u308a\u3057\u307e\u3059<\/li>\n<li>\u30aa\u30e9\u30f3\u30c0\u304b\u3089\u306e\u5831\u544a\u3067\u306f\uff0c60\u6b73\u307e\u3067\u306b\uff0c\u7537\u6027\u306e56\uff05\uff0c\u5973\u6027\u306e87%\u306b\u816b\u760d\u304c\u767a\u751f\u3057\u305f\u3068\u3044\u3046\u3053\u3068\u3067\u3059<\/li>\n<li>\u30d5\u30e9\u30f3\u30b9\u304b\u3089\u306e\u5831\u544a\u3067\u306f\uff0c70\u6b73\u307e\u3067\u306b\u764c\u304c\u767a\u751f\u3059\u308b\u78ba\u7387\u306f85%\u3068\u306e\u3053\u3068\u3067\u3059<\/li>\n<li>\u7279\u306b\u5973\u6027\u3067\u306f\uff0c\u4e73\u304c\u3093\uff0c\u7532\u72b6\u817a\u304c\u3093\uff0c\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5\u306e\u767a\u751f\u7387\u304c\u9ad8\u3044\u3068\u3055\u308c\u307e\u3059<\/li>\n<li>PTEN\u306f<strong><span style=\"color: #ff0000;\">\u81a0\u82bd\u816b glioblastoma \u306e\u539f\u56e0\u907a\u4f1d\u5b50<\/span><\/strong>\u306e\u4e00\u3064\u3068\u3057\u3066\u3082\u77e5\u3089\u308c\u3066\u3044\u307e\u3059<\/li>\n<li>\u307e\u305fPHTS\u306e\u306a\u304b\u306b\u306f\uff0c<span style=\"color: #ff0000;\"><strong>\u7cbe\u795e\u767a\u9054\u9045\u6ede\u3068\u81ea\u9589\u75c7<\/strong><\/span> autism \u3068\u3044\u3046\u7cbe\u795e\u75c7\u72b6\u3092\u51fa\u3059\u30bf\u30a4\u30d7\u3082\u3042\u308a\u307e\u3059<\/li>\n<\/ul>\n<h3>\u6587\u732e<\/h3>\n<ul>\n<li>Bubien V, et al.: High cumulative risks of cancer in patients with PTEN hamartoma tumour syndrome. J Med Genet 50: 255-263, 2013<\/li>\n<li>Nieuwenhuis MH, et al.: Cancer risk and genotype-phenotype correlations in PTEN hamartoma tumor syndrome. Fam Cancer. 2013 Aug 11. [Epub]<\/li>\n<li>Nowak DA, et al.: Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Clin Neurol Neurosurg 103: 105-110, 2001<\/li>\n<li>Nowak DA, et al.: Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 105: 137-145, 2002<\/li>\n<li>Robinson S, et al.: Cowden disease and Lhermitte-Duclos disease: characterization of a new phakomatosis. Neurosurgery 46: 371-383, 2000<\/li>\n<li>Takei H, et al.: Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. J Neurosurg 107: 137-142, 2007<\/li>\n<li>Wei G, et al.: Teaching neuroimages: MRI appearances of Lhermitte-Duclos disease. Neurology 80: e67-68, 2013<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5 \u00a0Lhermitte-Duclos disease \u5c0f\u8133\u7570\u5f62\u6210\u6027\u795e\u7d4c\u7bc0\u7d30\u80de\u816b \u00a0Dysplastic gangliocytoma of the cerebellum\u00a0 \u4e73\u5150\u304b\u308960\u4ee3\u307e\u3067\uff0c\u5e45\u5e83\u3044\u5e74 [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[5],"tags":[],"class_list":["post-4006","post","type-post","status-publish","format-standard","hentry","category-pediattumor"],"aioseo_notices":[],"aioseo_head":"\n\t\t<!-- All in One SEO 4.9.10 - aioseo.com -->\n\t<meta name=\"description\" content=\"\u30ec\u30eb\u30df\u30c3\u30c8\u30fb\u30c0\u30af\u30ed\u30b9\u75c5 Lhermitte-Duclos disease 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