Neurofibromatosis 2011: a report of the Children's Tumor Foundation annual meeting.

Neurofibromatosis 2011: a report of the Children's Tumor Foundation annual meeting.
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DOI:
10.1007/s00401-011-0905-0
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发表时间:
2012-03
影响因子:
12.7
通讯作者:
Ratner N
Ratner N
中科院分区:
医学1区
文献类型:
--
作者:
Kalamarides M;Acosta MT;Babovic-Vuksanovic D;Carpen O;Cichowski K;Evans DG;Giancotti F;Hanemann CO;Ingram D;Lloyd AC;Mayes DA;Messiaen L;Morrison H;North K;Packer R;Pan D;Stemmer-Rachamimov A;Upadhyaya M;Viskochil D;Wallace MR;Hunter-Schaedle K;Ratner N

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2011年儿童肿瘤基金会年会,神经纤维瘤病(NF)研究和临床社区的年度聚会,有330名与会者参加,他们讨论了新的信号通路整合到NF研究中,对散发性癌症中NF突变的赞赏,以及扩大临床前和临床议程。NF1,NF2和神经鞘瘤病共同影响美国约100,000人,并由不同基因的突变引起。NF1(神经纤维瘤和视神经通路胶质瘤)和NF2(神经鞘瘤、室管膜瘤和脑膜瘤)的良性肿瘤和神经鞘瘤病(神经鞘瘤)可导致显著的发病率,并且没有针对任何形式的NF的经证实的药物治疗。每种疾病都与引起发病的其他表现有关。本综述中描述的研究报告涵盖了基础科学、临床前测试和临床试验结果,并展示了基于科学家和临床医生之间的密切互动,在理解和治疗这些疾病方面取得的显著进展。
The 2011 annual meeting of the Children’s Tumor Foundation, the annual gathering of the neurofibromatosis (NF) research and clinical communities, was attended by 330 participants who discussed integration of new signaling pathways into NF research, the appreciation for NF mutations in sporadic cancers, and an expanding pre-clinical and clinical agenda. NF1, NF2, and schwannomatosis collectively affect approximately 100,000 persons in US, and result from mutations in different genes. Benign tumors of NF1 (neurofibroma and optic pathway glioma) and NF2 (schwannoma, ependymoma, and meningioma) and schwannomatosis (schwannoma) can cause significant morbidity, and there are no proven drug treatments for any form of NF. Each disorder is associated with additional manifestations causing morbidity. The research presentations described in this review covered basic science, preclinical testing, and results from clinical trials, and demonstrate the remarkable strides being taken toward understanding of and progress toward treatments for these disorders based on the close interaction among scientists and clinicians.