AIM To evaluate until what age children in families with retinoblastoma should be screened. METHODS A register based cohort (n= 685) study of Dutch retinoblastoma patients (1945-1998). The records of all familial hereditary retinoblastoma patients from 1945 were reviewed and the age at diagnosis and either they were screened from birth determined. RESULTS 75 patients had the familial heredi...

Studies on retinoblastoma have been at the heart of many of the landmark discoveries in cancer genetics over the past 35 years. However, these advances in the laboratory have had little effect on the treatment of children with retinoblastoma. One of the reasons for this has been the lack of preclinical models that recapitulated the genetic and histopathologic features of human retinoblastoma. I...

Actas Dermosifiliogr. 2009;100:615-34 626 some forms of brain tumor; although such outcomes have not been demonstrated for melanoma. The cells of retinoblastoma, melanoma and dysplastic nevus are derived from the neural crest. There are authors who postulate that patients with retinoblastoma and dysplastic nevus syndrome have increased genetic susceptibility to melanoma due to a primary anomaly...

The role of estimating the serum carcinoembryonic antigen (CEA) in the diagnosis and prognosis of retinoblastoma cases has been evaluated. Although the mean serum CEA titre in children with retinoblastoma was higher than that in control children (p = 0.01), the serum CEA level itself was found not to be a useful marker in the diagnosis of retinoblastoma. A significant correlation of serum CEA t...

Background. To determine clinical profile and management of retinoblastoma among children at Kinshasa in Democratic Republic of Congo. Patients and methods. The medical records of patients with a diagnosis of retinoblastoma seen at the University Hospital of Kinshasa from January 1985 till December 2005 were retrospectively reviewed. Demographic profile, clinical data, modes of treatment and ou...

The cell cycle regulator, retinoblastoma protein, is known to potentiate glucocorticoid receptor-activated transcription through the interaction of its pocket domain with the transcription coactivator, hBRM. We now show that glucocorticoid receptor-induced apoptosis is also dependent on both the retinoblastoma protein and hBRM. p107 and p130, which share extensive sequence homology with the poc...

PURPOSE To evaluate the clinical results of proton beam radiation therapy (PBRT) for treatment of retinoblastoma. METHODS Children with retinoblastoma who were treated with chemotherapy and focal treatment such as brachytherapy and thermotherapy but showed no response or developed recurrences later received PBRT. The PBRT strategy was designed to concentrate the radiation energy to the retino...

We report the first use in Hong Kong of molecular techniques to screen prenatally for retinoblastoma and review 17 cases of retinoblastoma seen at the Hong Kong Eye Hospital from 2001 to 2006. A pregnant couple whose first child had retinoblastoma requested prenatal screening for retinoblastoma during their second pregnancy in 2000. Whole RB1 coding gene sequencing was performed on peripheral b...

This manuscript focuses on high-risk factors of metastatic disease in retinoblastoma and evaluation of the current treatments of retinoblastoma. Presence of histopathologic high-risk factors is associated with a higher risk of local recurrence and systemic metastasis. Currently, globe-sparing therapies, including systemic chemotherapy, intra-arterial chemoreduction, intravitreal chemotherapy, f...

Retinoblastoma, an intraocular pediatric cancer, develops in the embryonic retina following biallelic loss of RB1. However, there is a wide range of genetic and epigenetic changes that can affect RB1 resulting in different clinical outcomes. In addition, other transformations, such as MYCN amplification, generate particularly aggressive tumors, which may or may not be RB1 independent. Recognizi...