Retinoblastoma: what is new in 2007-2008

Retinoblastoma: what is new in 2007-2008
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DOI:
10.1097/icu.0b013e328312975b
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发表时间:
2008-11-01
影响因子:
3.7
通讯作者:
Abramson, David H.
Abramson, David H.
中科院分区:
医学2区
文献类型:
--
作者:
Schefler, Amy C.;Abramson, David H.

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综述的目的本综述重点介绍了2007年6月至2008年5月发表的许多关于视网膜母细胞瘤的最重要的文章。在分子生物学,转化研究和临床报告的重大进展详细。最近的发现在视网膜母细胞瘤的分子生物学最重要的最近的发现包括证据为非整倍体和基因组不稳定性作为癌症的原因,而不是长期持有的Knudson的“两击”假说,证据表明视网膜瘤可能代表视网膜母细胞瘤的前驱病变之前收购的基因组不稳定性;以及水平中间神经元可能是小鼠敲除视网膜母细胞瘤的起源细胞并且在分化后能够克隆扩增的证据。转化研究也证明了在儿童中使用托泊替康和2-脱氧-D-葡萄糖的前景。最后,动脉内化疗对人眼内视网膜母细胞瘤的引入似乎是安全和有效的,并可能消除需要在许多patients.SummaryExciting在基础科学和新疗法的临床应用的新进展,继续出现这种罕见的疾病。我们预计,由于临床和实验室的新发现,在发达国家,即使是晚期眼内视网膜母细胞瘤的局部控制率也将很快达到100%。
Purpose of reviewThis review highlights many of the most important articles published from June 2007 to May 2008 on retinoblastoma. Significant advances in molecular biology, translational research, and clinical reports are detailed.Recent findingsThe most significant recent findings in the molecular biology of retinoblastoma include the evidence for aneuploidy and genomic instability as cancer causes rather than the long-held Knudson's 'two-hit' hypothesis; the evidence that retinoma may represent a precursor lesion for retinoblastoma prior to the acquisition of genomic instability; and the evidence that a horizontal interneuron may be the cell of origin in murine knockout retinoblastoma and may be capable of clonal expansion after differentiation. Translational studies also demonstrate promise for the use of topotecan and 2-deoxy-D-glucose in children. Finally, the introduction of intraarterial chemotherapy for human intraocular retinoblastoma appears to be safe and effective and may eliminate the need for enucleation in many patients.SummaryExciting new advances in both the basic science and clinical applications of new therapies continue to emerge for this rare disease. We expect that local control rates even for advanced intraocular retinoblastoma will soon reach 100% in the developed world as a result of new findings in the clinic and in the laboratory.