BACKGROUND The transcriptional co-activator, TAZ, is an important effector of the Hippo pathway and is critical for the development of human cancers. However, the expression and prognostic significance of TAZ in retinoblastoma is currently unclear. METHODS TAZ expression was examined in 43 retinoblastoma samples by immunohistochemistry. The relationship between TAZ expression and the clinicop...

Mutations of the retinoblastoma gene are known to cause both nonhereditary and hereditary forms of retinoblastoma. Most patients with hereditary retinoblastoma have bilateral disease. Hereditary predisposition to retinoblastoma is caused by a germline mutation at the retinoblastoma gene locus (RB1) and transmitted as an autosomal dominant trait with 90% penetrance. Three quarters of these alter...

AIM The aim of this study was to determine the (time trends in) incidence and survival of hereditary (familial and sporadic) and non-hereditary retinoblastoma for male and female patients born in the Netherlands between 1862 and 1995. METHOD The national retinoblastoma register was updated and now consists of 955 patients. The missing dates of death were obtained from the municipal registers ...

The objective of this paper was to identify hub genes and pathways associated with retinoblastoma using centrality analysis of the co-expression network and pathway-enrichment analysis. The co-expression network of retinoblastoma was constructed by weighted gene co-expression network analysis (WGCNA) based on differentially expressed (DE) genes, and clusters were obtained through the molecular ...

OBJECTIVE Little is known about the incidence of secondary neoplasms among survivors of retinoblastoma in Japan. The objective of our study was to analyze the cumulative incidence rate of secondary neoplasms following retinoblastoma and to investigate the risk factors of developing secondary neoplasms. METHODS We conducted a retrospective cohort study of 754 retinoblastoma patients who visite...

There is considerable debate about the significance of choroidal invasion of retinoblastoma with regard to metastatic disease. The charts of patients with retinoblastoma were reviewed over a 17 year period to determine the frequency of histopathological choroidal invasion of retinoblastoma and its risk for eventual metastatic disease. Sixty seven of 289 eyes (23%) enucleated for retinoblastoma ...

1Ophthalmologist, Medical Doctor specializing in ocular oncology, Ophtalmology Department at Hospital A.C.Camargo, São Paulo, SP, Brazil 2Medical Doctor specialized in Pediatrics, Pediatrics Department, Hospital A.C.Camargo, São Paulo, SP, Brazil 3Ophthalmologist, Ophtalmology Department at Hospital A.C.Camargo, São Paulo, SP, Brazil 4Ophthalmologist, Head of the Ophtalmology Department at Hosp...

Retinoblastoma, the most common intraocular malignant tumor in children, is characterized by the loss of both functional alleles of RB1 gene, which however alone cannot maintain malignant characteristics of retinoblastoma cells. Nevertheless, the investigation of other molecular aberrations such as matrix metalloproteinases (MMPs) and miRNAs is still lacking. In this study, we demonstrate that ...

UHRF1 (ubiquitin-like with PHD and RING finger domains 1) is a critical regulator for DNA methylation, and its frequent overexpression in human cancers has been associated with tumor-promoting effects. However, whether the overexpressed UHRF1 contributes to the establishment and maintenance of tumor methylomes and whether this process can affect the tumorigenesis remain unclear. In this study, ...

Chemotherapy combined with laser therapy and cryotherapy has improved the ocular salvage rate for children with bilateral retinoblastoma. However, children with late-stage disease often experience recurrence shortly after treatment. To improve the vision salvage rate in advanced bilateral retinoblastoma, we have developed and characterized two new rodent models of retinoblastoma for screening c...