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Telomere elongation mechanisms by ATRX/DRXX gene alterations and their clinical application

Research Project

Project/Area Number 25861670
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Pediatric surgery
Research InstitutionHiroshima University

Principal Investigator

ONITAKE Yoshiyuki  広島大学, 大学病院, 病院助教 (10448269)

Project Period (FY) 2013-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywords神経芽腫 / テロメア / ATRX / DRXX / 伸長 / 変異 / 悪性度 / 予後 / ATRX遺伝子 / 年長児
Outline of Final Research Achievements

In neuroblastomas with elongated telomeres by ALT (alaternative lengthening of telomere) mechanism, telomere signals by FISH were heterogeneous. In these 24 tumors, 16 had ATRX mutations, 6 had ATRX deletions, and the remaining 2 had DRXX mutations. Clinically, the children with such tumors were advanced stage ones without MYCN amplification except for one case. These tumor showed chemotherapeutic resistant and poor outcome significantly. Such ALT activated neuroblastoma is rare but a discriminate tumor group with poor prognosis.

Report

(3 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • Research Products

    (19 results)

All 2014 2013

All Journal Article (4 results) (of which Peer Reviewed: 4 results,  Open Access: 3 results) Presentation (15 results)

  • [Journal Article] Clinical features of ATRX or DAXX mutated neuroblastoma2014

    • Author(s)
      Kurihara S, Hiyama E, Onitake Y, Yamaoka E, Hiyama K
    • Journal Title

      Journal of Pediatric Surgery

      Volume: 49 Issue: 12 Pages: 1835-1838

    • DOI

      10.1016/j.jpedsurg.2014.09.029

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Detection of MYCN amplification using blood plasma: noninvasive therapy evaluation and prediction of prognosis in neuroblastoma2013

    • Author(s)
      Kojima M, Hiyama E, Fukuba I, Yamaoka E, Ueda Y, Onitake Y, Kurihara S, Sueda T
    • Journal Title

      Pediatric Surgery International

      Volume: 29 Issue: 11 Pages: 1139-1145

    • DOI

      10.1007/s00383-013-3374-9

    • Related Report
      2013 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] A cisplatin plus pirarubicin-based JPLT2 chemotherapy for hepatoblastoma: experience and future of the Japanese Study Group for Pediatric Liver Tumor (JPLT)2013

    • Author(s)
      Hiyama E, Ueda Y, Onitake Y, Kurihara S, Watanabe K, Hishiki T, Tajiri T, Ida K, Yano M, Kondo S, Oue T; Japanese Study Group for Pediatric Liver Tumor
    • Journal Title

      Pediatric Surgery International

      Volume: 29 Issue: 10 Pages: 1071-1075

    • DOI

      10.1007/s00383-013-3399-0

    • Related Report
      2013 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] 外傷性脾損傷後に発症した鼠径ヘルニアの1例2013

    • Author(s)
      鬼武 美幸, 檜山 英三, 小倉 薫, 上田 祐華
    • Journal Title

      小児外科

      Volume: 45 Pages: 1260-1262

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Presentation] Surgical managements for refractory neuroblastoma in young children2014

    • Author(s)
      Hiyama E, Kurihara S, Onitake Y, Ueda Y, Miki M, Kawaguchi H, Nakamura K, Kobayashi MK
    • Organizer
      BAPS61st Annual Congress
    • Place of Presentation
      Edinburgh, Scotland
    • Year and Date
      2014-07-22 – 2014-07-25
    • Related Report
      2014 Annual Research Report
  • [Presentation] Central venous catheter-related complications in children with malignancy2014

    • Author(s)
      .Kurihara S, Hiyama E, Onitake Y, Miki M, Nakamura K, Kawaguchi H, Kobayashi M
    • Organizer
      PAPS2014
    • Place of Presentation
      Calgary, Canada
    • Year and Date
      2014-05-25 – 2014-05-29
    • Related Report
      2014 Annual Research Report
  • [Presentation] Clinical feature of ATRX or DAXX mutated neuroblastoma2014

    • Author(s)
      Hiyama E, Kurihara S, Onitake Y, Yamaoka E, Fukuba I, Hiyama K
    • Organizer
      PAPS2014
    • Place of Presentation
      Calgary, Canada
    • Year and Date
      2014-05-25 – 2014-05-29
    • Related Report
      2014 Annual Research Report
  • [Presentation] Wnt Signaling and telomerase activation in hepatoblastoma2013

    • Author(s)
      Hiyama E, Ueda Y, Kamimatsuse A, Onitake Y, Ogura K, Hiyama K
    • Organizer
      AACR Annual Meeintg2013
    • Place of Presentation
      Walter E. Washington Convention Center(アメリカ ワシントン)
    • Related Report
      2013 Research-status Report
  • [Presentation] 2度の再発を来した脂肪芽腫の1例2013

    • Author(s)
      小倉 薫, 檜山 英三, 鬼武 美幸, 上田 祐華, 谷 守通
    • Organizer
      第54回中国四国小児がん研究会
    • Place of Presentation
      岩国医療センター(山口県岩国市)
    • Related Report
      2013 Research-status Report
  • [Presentation] 血中DNA を用いた神経芽腫の分子診断:予後予測と治療効果判定2013

    • Author(s)
      檜山 英三,児島 正人,河島 茉澄,上田 祐華,鬼武 美幸,上松瀬 新,小倉 薫,末田 泰二郎
    • Organizer
      第50回日本小児外科学会学術集会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Related Report
      2013 Research-status Report
  • [Presentation] 18 トリソミーに合併した肝芽腫の1 例―染色体異常を合併したJPLT 登録症例の検討を併せて―2013

    • Author(s)
      鬼武 美幸,檜山 英三,小倉 薫,上松瀬 新, 中村 和洋,古江 綾
    • Organizer
      第50回日本小児外科学会学術集会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Related Report
      2013 Research-status Report
  • [Presentation] 術後に心不全の改善を認めた静脈管開存症の1 例2013

    • Author(s)
      小倉 薫,檜山 英三,上松瀬 新,鬼武 美幸,上田 祐華
    • Organizer
      第50回日本小児外科学会学術集会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Related Report
      2013 Research-status Report
  • [Presentation] Survival, surgical respectability, and late effects in the hepatoblastoma patients treated by cisplatin plus pirarubicin(CITA) chemotherapy2013

    • Author(s)
      Hiyama E, Kamimatsuse A, Onitake Y, Ueda Y, Watanabe K, Hishiki T, Tajiri T, Ida K, Yano M, Kondo S, Oue T
    • Organizer
      ASCO Annual ’ 13 Meeting
    • Place of Presentation
      McComick Place(アメリカ シカゴ)
    • Related Report
      2013 Research-status Report
  • [Presentation] FURTHER STUDY: IDENTIFICATION OF NEW CANDIDATE GENES IN PROGRESSION OF HEPATOBLASTOMA USING GENOME-WIDE ASSOCIATION STUDY2013

    • Author(s)
      Hiyama E, Ueda Y, Onitake Y, Ida K, Ogura K, Kondo S, Kamijyo T, Watanabe K, Oue T, Hishiki T, Tajiri T, Horie H, Inoue T, Tanaka Y
    • Organizer
      45th Congress of the International Society of Pediatric Oncology
    • Place of Presentation
      Hong Kong Convention and Exhibition Centre(中国 香港)
    • Related Report
      2013 Research-status Report
  • [Presentation] 肺転移巣手術のタイミングに苦慮した高リスク肝芽腫の一例2013

    • Author(s)
      栗原 将, 鬼武 美幸, 小倉 薫, 上田 祐華, 檜山 英三, 世羅 康彦, 三木 瑞香, 中村 和洋, 小林 正夫
    • Organizer
      第2回せとうち小児がんセミナー
    • Place of Presentation
      ホテルグランヴィア岡山(岡山県岡山市)
    • Related Report
      2013 Research-status Report
  • [Presentation] 外傷性脾損傷後に発症した鼠径ヘルニアの1例2013

    • Author(s)
      鬼武 美幸, 檜山 英三, 小倉 薫, 上田 祐華
    • Organizer
      第29回日本小児外科学会秋季シンポジウム
    • Place of Presentation
      一橋大学一橋講堂(東京都千代田区)
    • Related Report
      2013 Research-status Report
  • [Presentation] 高用量シスプラチン療法後に腫瘍全摘を行った門脈浸潤を伴う肝芽腫の一例2013

    • Author(s)
      江藤 昌平, 古江 綾, 千々松 郁枝, 三木 瑞香, 世羅 康彦, 川口 浩史, 中村 和洋, 上田 祐華, 鬼武 美幸, 小倉 薫, 檜山 英三, 小林 正夫
    • Organizer
      第55回日本小児血液・がん学会学術集会
    • Place of Presentation
      ヒルトン福岡シーホーク(福岡県福岡市)
    • Related Report
      2013 Research-status Report
  • [Presentation] 腹腔内Desmoplastic small round cell tumorの1小児例2013

    • Author(s)
      斎藤 聡志, 松本 惇子, 坂田 園子, 千々松 郁枝, 三木 瑞香, 世羅 康彦, 川口 浩史, 中村 和洋, 上田 祐華, 鬼武 美幸, 小倉 薫, 檜山 英三, 小林 正夫
    • Organizer
      第55回日本小児血液・がん学会学術集会
    • Place of Presentation
      ヒルトン福岡シーホーク(福岡県福岡市)
    • Related Report
      2013 Research-status Report
  • [Presentation] Wilms腫瘍を合併した縦隔奇形腫の1例2013

    • Author(s)
      小倉 薫, 檜山 英三, 鬼武 美幸, 上田 祐華, 千々松 郁枝, 世羅 康彦, 小林 正夫
    • Organizer
      第55回日本小児血液・がん学会学術集会
    • Place of Presentation
      ヒルトン福岡シーホーク(福岡県福岡市)
    • Related Report
      2013 Research-status Report

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Published: 2014-07-25   Modified: 2019-07-29  

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