Retinoblastoma: what is new in 2007-2008.
ABSTRACT This 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.
The 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.
Exciting 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.
- SourceAvailable from: Sidney Fu[Show abstract] [Hide abstract]
ABSTRACT: Retinoblastoma (RB) is a children's ocular cancer caused by mutated retinoblastoma 1 (Rb1) gene on both alleles. Rb1 and other related genes could be regulated by microRNAs (miRNA) via complementarily pairing with their target sites. MicroRNA-21 (miR-21) possesses the oncogenic potential to target several tumor suppressor genes, including PDCD4, and regulates tumor progression and metastasis. However, the mechanism of how miR-21 regulates PDCD4 is poorly understood in RB. We investigated the expression of miRNAs in RB cell lines and identified that miR-21 is one of the most deregulated miRNAs in RB. Using qRT-PCR, we verified the expression level of several miRNAs identified by independent microarray assays, and analyzed miRNA expression patterns in three RB cell lines, including Weri-Rb1, Y79 and RB355. We found that miR-19b, -21, -26a, -195 and -222 were highly expressed in all three cell lines, suggesting their potential role in RB tumorigenesis. Using the TargetScan program, we identified a list of potential target genes of these miRNAs, of which PDCD4 is one the targets of miR-21. In this study, we focused on the regulatory mechanism of miR-21 on PDCD4 in RB. We demonstrated an inverse correlation between miR-21 and PDCD4 expression in Weri-Rb1 and Y79 cells. These data suggest that miR-21 down-regulates Rb1 by targeting PDCD4 tumor suppressor. Therefore, miR-21 could serve as a therapeutic target for retinoblastoma.Journal of Cancer. 01/2014; 5(9):804-12.
- [Show abstract] [Hide abstract]
ABSTRACT: Hereditary retinoblastoma (Rb) survivors have increased risk of subsequent malignant neoplasms (SMNs). Previous studies reported elevated radiotherapy (RT) -related SMN risks, but less is known about chemotherapy-related risks.Journal of Clinical Oncology 09/2014; · 17.88 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Abstract Introduction: The purpose of this paper is to perform a systematic review of the published literature on complications of intra-arterial chemothrapy (IAC) for the treatment of retinoblastoma.Seminars in Ophthalmology 11/2014; 29(5-6):429-33. · 1.20 Impact Factor