medulloblastoma

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髄芽腫

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/06/27 18:40:10」(JST)

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英文文献

  • Downregulation of microRNA-431 by human interferon-β inhibits viability of medulloblastoma and glioblastoma cells via upregulation of SOCS6.
  • Tanaka T1, Arai M2, Jiang X2, Sugaya S1, Kanda T2, Fujii K3, Kita K1, Sugita K4, Imazeki F2, Miyashita T5, Kaneda A1, Yokosuka O2.Author information 1Department of Molecular Oncology, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.2Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.3Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.4Faculty of Education, Chiba University, Chiba 263-8522, Japan.5Department of Molecular Genetics, Kitasato University Graduate School of Medical Sciences, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan.AbstractmiRNAs are small non-coding RNAs that inhibit gene expression by cleaving or hindering the translation of target mRNAs. In this study, we focused on miR-431, which mediated inhibition of cell viability by human interferon-β (HuIFN-β). We aimed to demonstrate an antineoplastic effect of HuIFN-β via miR-431 expression against medulloblastoma and glioblastoma, because HuIFN-β is frequently used in adjuvant therapy of these tumors. Addition of HuIFN-β to medulloblastoma and glioblastoma cells reduced viability, significantly decreased miR-431 expression, upregulated expression of SOCS6 (putative miR-431 target genes) and inhibited Janus kinase (JAK) 1 and signal transducer and activator of transcription (STAT) 2. The mitogen-activated protein kinase (MAPK) pathway, but not the phosphoinositide 3-kinase (PI3K)-Akt pathway, was downregulated in medulloblastoma cells, whereas the PI3K-Akt pathway, but not the MAPK pathway, was downregulated in glioblastoma cells. Addition of HuIFN-β and transient transfection with miR-431 to medulloblastoma and glioblastoma cells did not reduce viability, downregulated expression of SOCS6, and concomitantly activated the JAK1 and STAT2. We propose that, in medulloblastoma and glioblastoma cells, HuIFN-β decreases miR-431 expression and upregulates SOCS6 expression, and consequently inhibit cell proliferation by suppressing the JAK-STAT signaling pathway.
  • International journal of oncology.Int J Oncol.2014 May;44(5):1685-90. doi: 10.3892/ijo.2014.2317. Epub 2014 Feb 28.
  • miRNAs are small non-coding RNAs that inhibit gene expression by cleaving or hindering the translation of target mRNAs. In this study, we focused on miR-431, which mediated inhibition of cell viability by human interferon-β (HuIFN-β). We aimed to demonstrate an antineoplastic effect of HuIFN-β vi
  • PMID 24584142
  • High-dose busulfan-thiotepa with autologous stem cell transplantation followed by posterior fossa irradiation in young children with classical or incompletely resected medulloblastoma.
  • Bergthold G1, Kababri ME, Varlet P, Dhermain F, Sainte-Rose C, Raquin MA, Kieffer V, Goma G, Grill J, Valteau-Couanet D, Dufour C.Author information 1Department of Paediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France; American Memorial Hospital, CHU de Reims, Reims, France.AbstractBACKGROUND: The aim of the study is to evaluate the outcome of young children with high risk localized medulloblastomas (newly diagnosed classical or incompletely resected) treated by high-dose busulfan-thiotepa with autologous stem cell rescue (ASCT) followed by focal radiation therapy (RT).
  • Pediatric blood & cancer.Pediatr Blood Cancer.2014 May;61(5):907-12. doi: 10.1002/pbc.24954. Epub 2014 Jan 28.
  • BACKGROUND: The aim of the study is to evaluate the outcome of young children with high risk localized medulloblastomas (newly diagnosed classical or incompletely resected) treated by high-dose busulfan-thiotepa with autologous stem cell rescue (ASCT) followed by focal radiation therapy (RT).PROCEDU
  • PMID 24470384
  • Transitioning from genotypes to epigenotypes: Why the time has come for medulloblastoma epigenomics.
  • Batora NV1, Sturm D1, Jones DT1, Kool M1, Pfister SM2, Northcott PA3.Author information 1Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany.2Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany; Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Im Neuenheimer Feld 430, Heidelberg 69120, Germany.3Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany. Electronic address: p.northcott@dkfz-heidelberg.de.AbstractRecent advances in genomic technologies have allowed for tremendous progress in our understanding of the biology underlying medulloblastoma, a malignant childhood brain tumor. Consensus molecular subgroups have been put forth by the pediatric neuro-oncology community and next-generation genomic studies have led to an improved description of driver genes and pathways somatically altered in these subgroups. In contrast to the impressive pace at which advances have been made at the level of the medulloblastoma genome, comparable studies of the epigenome have lagged behind. Complementary data yielded from genomic sequencing and copy number profiling have verified frequent targeting of chromatin modifiers in medulloblastoma, highly suggestive of prominent epigenetic deregulation in the disease. Past studies of DNA methylation-dependent gene silencing and microRNA expression analyses further support the concept of medulloblastoma as an epigenetic disease. In this Review, we aim to summarize the key findings of past reports pertaining to medulloblastoma epigenetics as well as recent and ongoing genomic efforts linking somatic alterations of the genome with inferred deregulation of the epigenome. In addition, we predict what is on the horizon for medulloblastoma epigenetics and how aberrant changes in the medulloblastoma epigenome might serve as an attractive target for future therapies.
  • Neuroscience.Neuroscience.2014 Apr 4;264C:171-185. doi: 10.1016/j.neuroscience.2013.07.030. Epub 2013 Jul 20.
  • Recent advances in genomic technologies have allowed for tremendous progress in our understanding of the biology underlying medulloblastoma, a malignant childhood brain tumor. Consensus molecular subgroups have been put forth by the pediatric neuro-oncology community and next-generation genomic stud
  • PMID 23876321

和文文献

  • 小脳性無言症の障害機序と予後に関する一考察 : 小脳髄芽腫術後に無言症を呈した児の言語訓練経過から
  • 藤田 隼平,川崎 聡大,古西 隆之
  • とやま発達福祉学年報 4, 29-33, 2013-05-31
  • 小脳髄芽腫術後に小脳性無言症を呈した7歳の男児に対して言語訓練を実施した。本症例は発話表出のみならず全般的なコミュニケーション機能の低下と発動性の低下を呈したことと、水頭症や白質周囲の浮腫的病変から、視床や補足運動野の関与が考えられた。小脳性無言症から回復を見せた8か月後においても後遺症と思われる知的発達遅滞を認めたことからも、小脳性無言症を呈した児に関しては主徴候である表出面だけでなく、中長期的 …
  • NAID 120005290255
  • In vitro stemness characterization of radio-resistant clones isolated from a medulloblastoma cell line ONS-76
  • Sun Lue,Moritake Takashi,Zheng Yun-Wen,Suzuki Kenshi,Gerelchuluun Ariungerel,Hong Zhengshan,Zenkoh Junko,Taniguchi Hideki,Tsuboi Koji,盛武 敬,坪井 康次
  • Journal of radiation research 54(1), 61-69, 2013-01-00
  • … One-third of patients with medulloblastoma die due to recurrence after various treatments including radiotherapy. … Although it has been postulated that cancer stem-like cells are radio-resistant and play an important role in tumor recurrence, the "stemness" of medulloblastoma cells surviving irradiation has not yet been elucidated. … Using a medulloblastoma cell line ONS-76, cells that survived gamma irradiation were investigated on their "stemness" in vitro. …
  • NAID 120005246509
  • 44. Medulloblastoma加療後に発症した巨大卵巣腫瘍の1例(一般演題,第47回日本小児外科学会関東甲信越地方会)
  • 神保 教広,瓜田 泰久,高安 肇,新開 統子,藤代 準,五藤 周,上杉 達,坂元 直哉,佐々木 理人,増本 幸二
  • 日本小児外科学会雑誌 48(7), 1083-1084, 2012-12-20
  • NAID 110009562310

関連リンク

ここでは、その発生に年齢、部位が強く関連しているふたつの腫瘍を独特な腫瘍として取り上げる。 ひとつは medulloblastoma である。 本腫瘍の発生は、その 70% は 16 歳以下、そのピークは 7 歳頃である。 男性にやや多い傾向が ...
Medulloblastoma. First used by Bailey and Cushing in 1925[1] , the term medulloblastoma described a series of tumors found in the cerebella of children. ... Raj D Sheth, MD Professor, Mayo College of Medicine; Chief ...

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★リンクテーブル★
国試過去問093A079
リンク元脳腫瘍」「髄芽腫」「medullomyoblastoma
拡張検索desmoplastic medulloblastoma」「childhood medulloblastoma

093A079」

  [★]

  • 放射線感受性の高い脳腫瘍はどれ?
  • a. (1)(2)
  • b. (1)(5)
  • c. (2)(3)
  • d. (3)(4)
  • e. (4)(5)

脳腫瘍」

  [★]

brain tumor, cerebral tumor
脳新生物 brain neoplasm
頭蓋内腫瘍

概念

分類

疫学

腫瘍別発生頻度

腫瘍別発生頻度 小児 成人
神経膠腫 33% 星状細胞腫 髄膜腫
髄膜腫 22% 髄芽腫 膠芽腫
下垂体腺腫 15% 頭蓋咽頭腫 下垂体腺腫
神経鞘腫 9% 胚細胞腫 神経鞘腫
頭蓋咽頭腫 5% 上衣腫 転移性脳腫瘍

YN.J.188

部位 種類 小児 成人
頭蓋骨 頭蓋骨腫瘍
大脳半球 神経膠腫  
髄膜腫  
松果体 胚細胞腫  
小脳半球 星細胞腫  
血管芽腫  
小脳虫部 髄芽腫  
第四脳室 上衣腫  
鞍上部・
視交叉部・
下垂体部
頭蓋咽頭腫  
視神経膠腫  
胚細胞腫  
下垂体腺腫  
髄膜腫  
小脳橋角部 聴神経鞘腫  
脳幹部 神経膠腫

小児の脳腫瘍

SCN.173
  • 腫瘍:星細胞腫、髄芽腫、頭蓋咽頭腫、胚細胞腫、上衣腫の順に多い。
  • 部位:1歳まではテント上、2-7歳まではテント下、8-15歳まではテント上に多い。

放射線感受性

SCN.173
  • (高い)髄芽腫、胚細胞腫 > (低い)神経膠腫

転移性脳腫瘍

  • 肺癌(約半数)、乳癌、消化器癌、腎癌
  • 頻度:肺癌>乳癌>胃・腸癌 (SCN.173)

石灰化が見られる脳腫瘍

  • 乏突起膠腫:CT上、低吸収領域の中に石灰化がみられる。(SCN.173)
  • 上衣腫:CT:(単純CT)等~低吸収、(造影CT)中~強度の増強。小嚢胞や壊死、石灰化を認める
  • 髄膜腫:腫瘍の一部石灰化が少なからず見られる
  • 頭蓋咽頭腫:小児において石灰沈着が高頻度にみられる。

嚢胞性腫瘍

参考

  • 1. がんサポートセンター 脳腫瘍
[display]http://www.gsic.jp/cancer/cc_19/hc/02.html
  • 1-1. がんサポートセンター 脳腫瘍 フローチャート
[display]http://www.gsic.jp/cancer/cc_19/hc/cc_19_021.html
  • 2. 脳腫瘍 治療法ガイドライン [総論]
[display]http://www.ebm.jp/disease/brain/07noshuyo1/guide.html
  • 3. 資料
[display]http://ganjoho.ncc.go.jp/public/cancer/data/brain_adult.html
  • 4. 資料
[display]http://ganjoho.ncc.go.jp/public/cancer/data/brain_child.html
  • 5. 小児脳腫瘍
[display]http://www.geocities.jp/ululu_o_ululu/report-11.html

国試



髄芽腫」

  [★]

medulla 'bone marrow'
medulloblastoma
髄芽細胞腫

疫学

SCN.174
  • 男女=1.7:1
  • まれに20歳以降
  • 2/3は15歳以下、30%が15-35歳 (ATP.488)

好発部位

SCN.174
  • 小児(5-10歳)で小脳虫部。時に小脳半球

病理

  • 小型円型異型細胞が密に増殖。クロマチンに富み、核は類円形。細胞質は乏しい。核分裂増は多い
  • rosettes様配列(花環状配列, Homer-Wright型ロゼット)
  • N-CAM:強陽性 ← 神経細胞由来?
  • GFAP±
  • MIB-1 LI:約20%
  • (他のembryonal tumorと区別するにはさらにマーカーが必要)
  • WHO grade IV

CNS tumor: embryonal tumor

  • medulloblastomaWHO grades of CNS tumor = IV
  • CNS primitive neuroectodermal tumor PNET: WHO grades of CNS tumor = IV
  • atypical teratoid tumor/atypical rhabdoid tumor: WHO grades of CNS tumor = IV

進展・転移

  • 周囲組織に浸潤傾向が強い
  • くも膜下腔に播種しやすい

症状

  • 頭痛、嘔吐(噴出性の嘔吐)、体幹運動失調(転びやすい)
  • 乳幼児では水頭症で初発することがある。 ← 小脳虫部に原発して第四脳室を閉塞しうることと、乳幼児では前述の症状を訴えにくいことによる

検査

MRI

  • T1:低信号
  • T2:等信号~高信号
  • T1強調画像Gd造影:著明に増強

治療

SCN.175
  • 方針:手術療法で全摘出を目指す。3歳以上には放射線療法と化学療法、3歳未満には化学療法(メトトレキサート)を優先。
  • 手術療法
  • 放射線療法:喉頭蓋窩、全脳、全脊髄 ← 放射線感受性が高い腫瘍
  • 化学療法

予後

  • 5年生存率:50%以下 (SCN.175)
  • 10年生存率:40-50%

参考

  • 1. CT - 写真
[display]http://wiki.cns.org/wiki/images/thumb/6/60/MedulloMRI.jpg/250px-MedulloMRI.jpg
  • 2. 組織写真
[display]http://frontalcortex.com/2%E2%96%A0%E2%96%A0page=oll&topic=24&qid=898
  • 3.
[display]http://www.cancer.net/patient/Cancer+Types/Medulloblastoma+-+Childhood/ci.Medulloblastoma%2B-%2BChildhood.printer
  • 4. [charged] Epidemiology, treatment, and prognosis of medulloblastoma - uptodate [1]


medullomyoblastoma」

  [★]

髄筋芽腫髄筋芽細胞腫

childhood medulloblastomadesmoplastic medulloblastomamedulloblastoma

desmoplastic medulloblastoma」

  [★]

線維形成性髄芽腫

childhood medulloblastomamedulloblastomamedullomyoblastoma

childhood medulloblastoma」

  [★]

小児髄芽腫

desmoplastic medulloblastomamedulloblastomamedullomyoblastoma



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