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ASCO2017：炎症性肌纤维母细胞肿瘤的临床和基因组资料

2017-06-06 MedSci MedSci原创

炎症性肌纤维母细胞肿瘤基因组
关注

炎症性肌纤维母细胞肿瘤（IMT）是形态学上穿越肿瘤和炎症界限的肿瘤。许多儿童和年轻成年人患者出现全身症状，肿瘤显示局部侵袭性甚至转移。IMTs由具成簇的有明显的淋巴浆细胞浸润的肌纤维母细胞梭形细胞的组成。

炎症性肌纤维母细胞肿瘤（IMT）是形态学上穿越肿瘤和炎症界限的肿瘤。许多儿童和年轻成年人患者出现全身症状，肿瘤显示局部侵袭性甚至转移。IMTs由具成簇的有明显的淋巴浆细胞浸润的肌纤维母细胞梭形细胞的组成。

研究人员采用混合捕获，配对末端NGS测序方法和定制分析，对30例患者的IMTs DNA和RNA进行综合基因组分析。包括23例年龄在7〜72岁的儿童或青少年（<30岁）。10例患者中，3例侵袭性转移，5例多灶性，2例局部浸润。2例中有上皮样物质形态学表明ALK-RANBP2融合。13例患者检测到ALK重排，以7种不同方式融合。在23例患者中进行ALK IHC或FISH，结果均与基因组一致。在21例患者中检测到22个基因融合，4个肿瘤具有ROS1-TFG融合体，2个具有NTRK3-ETV6融合体。3分别在JAK1-PML，PDGFRB-NOTCH1和MUTYH-TESK2中有新的融合。JAK1中的完整酪氨酸激酶结构域（TKD）在重排中被转置到PML中的启动子卷曲螺旋基序上。虽然TKDs在PDGRB和TESK2重排中保留，但其伴侣的功能作用尚未得到阐明。30岁以下（20/23）患者的重排率高于30岁以上（3/7）的。所有10种侵袭性肿瘤均检测到重排。

研究表明IMT主要在年龄较小的年龄组发生。在年龄较小的患者以及更具侵袭性的肿瘤中，更容易观察到基因重排。考虑到损伤的血液学成分，以及新型酪氨酸激酶重排的存在，仍需进一步研究以阐明其中机制，因此与造血赘生物（如JAK1等）相关基因的改变将成为新的关注点。

原始出处：

Sugganth Daniel, Laurie M. Gay, Jo-Anne Vergilio, et.al. A clinical and genomic profile of inflammatory myofibroblastic tumors. 2017 ASCO Annual Meeting. June 3 2017.

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    2017-08-01 okhuali

    #肌纤维#

    0

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    2018-03-20 quxin068

    #ASC#

    0

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    2018-03-23 12498e0fm90暂无昵称

    #纤维母细胞#

    0

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    2017-06-08 12498dd9m15暂无昵称

    #炎症性#

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=2078012, encodeId=357d20e8012a1, content=<a href='/topic/show?id=8a8580960a5' target=_blank style='color:#2F92EE;'>#肌纤维#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80960, encryptionId=8a8580960a5, topicName=肌纤维)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ea6321, createdName=okhuali, createdTime=Tue Aug 01 00:11:00 CST 2017, time=2017-08-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1938704, encodeId=836d1938e04f3, content=<a href='/topic/show?id=1c292818fd' target=_blank style='color:#2F92EE;'>#ASC#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2818, encryptionId=1c292818fd, topicName=ASC)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=74808, createdName=quxin068, createdTime=Tue Mar 20 10:11:00 CST 2018, time=2018-03-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1766613, encodeId=13cc1e66613ae, content=<a href='/topic/show?id=d2c6e724888' target=_blank style='color:#2F92EE;'>#纤维母细胞#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77248, encryptionId=d2c6e724888, topicName=纤维母细胞)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=342437842399, createdName=12498e0fm90暂无昵称, createdTime=Fri Mar 23 00:11:00 CST 2018, time=2018-03-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1547047, encodeId=21fd154e04767, content=<a href='/topic/show?id=4ef26e01766' target=_blank style='color:#2F92EE;'>#炎症性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=67017, encryptionId=4ef26e01766, topicName=炎症性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=09e813968720, createdName=12498dd9m15暂无昵称, createdTime=Thu Jun 08 05:11:00 CST 2017, time=2017-06-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207531, encodeId=206f20e53129, content=学习了新观点, beContent=null, objectType=article, channel=null, level=null, likeNumber=93, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib260FNcic8ThYQJjaU2fLIb1yNqibkbdJs7gVIDr9xJyYeiapArSMjn3W61bPJtpIazSdAxRaFEOKYgfQjX4achN2d/0, createdBy=68472058812, createdName=日月生辉, createdTime=Wed Jun 07 06:47:22 CST 2017, time=2017-06-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207367, encodeId=f40720e36786, content=学习了新知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=94, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Tue Jun 06 18:23:07 CST 2017, time=2017-06-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207352, encodeId=186420e352dd, content=学习了已经，谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=97, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib06iaV3D9Tg5K4BGdu4COj6QWFqgMd3xnWDdgLZKUGI32veZvO2qFAQvpYr1eMbUOTqMvlZWc3MsugtFMHzxDwial/0, createdBy=88731939304, createdName=圣艮山, createdTime=Tue Jun 06 14:38:25 CST 2017, time=2017-06-06, status=1, ipAttribution=)]
    2017-06-07 日月生辉

    学习了新观点

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2078012, encodeId=357d20e8012a1, content=<a href='/topic/show?id=8a8580960a5' target=_blank style='color:#2F92EE;'>#肌纤维#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80960, encryptionId=8a8580960a5, topicName=肌纤维)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ea6321, createdName=okhuali, createdTime=Tue Aug 01 00:11:00 CST 2017, time=2017-08-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1938704, encodeId=836d1938e04f3, content=<a href='/topic/show?id=1c292818fd' target=_blank style='color:#2F92EE;'>#ASC#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2818, encryptionId=1c292818fd, topicName=ASC)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=74808, createdName=quxin068, createdTime=Tue Mar 20 10:11:00 CST 2018, time=2018-03-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1766613, encodeId=13cc1e66613ae, content=<a href='/topic/show?id=d2c6e724888' target=_blank style='color:#2F92EE;'>#纤维母细胞#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77248, encryptionId=d2c6e724888, topicName=纤维母细胞)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=342437842399, createdName=12498e0fm90暂无昵称, createdTime=Fri Mar 23 00:11:00 CST 2018, time=2018-03-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1547047, encodeId=21fd154e04767, content=<a href='/topic/show?id=4ef26e01766' target=_blank style='color:#2F92EE;'>#炎症性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=67017, encryptionId=4ef26e01766, topicName=炎症性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=09e813968720, createdName=12498dd9m15暂无昵称, createdTime=Thu Jun 08 05:11:00 CST 2017, time=2017-06-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207531, encodeId=206f20e53129, content=学习了新观点, beContent=null, objectType=article, channel=null, level=null, likeNumber=93, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib260FNcic8ThYQJjaU2fLIb1yNqibkbdJs7gVIDr9xJyYeiapArSMjn3W61bPJtpIazSdAxRaFEOKYgfQjX4achN2d/0, createdBy=68472058812, createdName=日月生辉, createdTime=Wed Jun 07 06:47:22 CST 2017, time=2017-06-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207367, encodeId=f40720e36786, content=学习了新知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=94, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Tue Jun 06 18:23:07 CST 2017, time=2017-06-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207352, encodeId=186420e352dd, content=学习了已经，谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=97, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib06iaV3D9Tg5K4BGdu4COj6QWFqgMd3xnWDdgLZKUGI32veZvO2qFAQvpYr1eMbUOTqMvlZWc3MsugtFMHzxDwial/0, createdBy=88731939304, createdName=圣艮山, createdTime=Tue Jun 06 14:38:25 CST 2017, time=2017-06-06, status=1, ipAttribution=)]
    2017-06-06 Y—xianghai

    学习了新知识

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=2078012, encodeId=357d20e8012a1, content=<a href='/topic/show?id=8a8580960a5' target=_blank style='color:#2F92EE;'>#肌纤维#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80960, encryptionId=8a8580960a5, topicName=肌纤维)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ea6321, createdName=okhuali, createdTime=Tue Aug 01 00:11:00 CST 2017, time=2017-08-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1938704, encodeId=836d1938e04f3, content=<a href='/topic/show?id=1c292818fd' target=_blank style='color:#2F92EE;'>#ASC#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2818, encryptionId=1c292818fd, topicName=ASC)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=74808, createdName=quxin068, createdTime=Tue Mar 20 10:11:00 CST 2018, time=2018-03-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1766613, encodeId=13cc1e66613ae, content=<a href='/topic/show?id=d2c6e724888' target=_blank style='color:#2F92EE;'>#纤维母细胞#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77248, encryptionId=d2c6e724888, topicName=纤维母细胞)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=342437842399, createdName=12498e0fm90暂无昵称, createdTime=Fri Mar 23 00:11:00 CST 2018, time=2018-03-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1547047, encodeId=21fd154e04767, content=<a href='/topic/show?id=4ef26e01766' target=_blank style='color:#2F92EE;'>#炎症性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=67017, encryptionId=4ef26e01766, topicName=炎症性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=09e813968720, createdName=12498dd9m15暂无昵称, createdTime=Thu Jun 08 05:11:00 CST 2017, time=2017-06-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207531, encodeId=206f20e53129, content=学习了新观点, beContent=null, objectType=article, channel=null, level=null, likeNumber=93, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib260FNcic8ThYQJjaU2fLIb1yNqibkbdJs7gVIDr9xJyYeiapArSMjn3W61bPJtpIazSdAxRaFEOKYgfQjX4achN2d/0, createdBy=68472058812, createdName=日月生辉, createdTime=Wed Jun 07 06:47:22 CST 2017, time=2017-06-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207367, encodeId=f40720e36786, content=学习了新知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=94, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Tue Jun 06 18:23:07 CST 2017, time=2017-06-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=207352, encodeId=186420e352dd, content=学习了已经，谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=97, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/oLAjfB7s1ib06iaV3D9Tg5K4BGdu4COj6QWFqgMd3xnWDdgLZKUGI32veZvO2qFAQvpYr1eMbUOTqMvlZWc3MsugtFMHzxDwial/0, createdBy=88731939304, createdName=圣艮山, createdTime=Tue Jun 06 14:38:25 CST 2017, time=2017-06-06, status=1, ipAttribution=)]
    2017-06-06 圣艮山

    学习了已经，谢谢

    0

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