PURPOSE Intra-arterial delivery of chemotherapeutic agents offers a new and exciting opportunity for the treatment of advanced intraocular retinoblastoma. It allows local delivery of relatively high doses of chemotherapy agents while bypassing general blood circulation. For this reason, this study was undertaken to revisit some of the FDA-approved drugs for the treatment of retinoblastoma. ME...

Retinoblastoma is a childhood cancer of the developing retina that begins in utero and is diagnosed in the first years of life. Biallelic RB1 gene inactivation is the initiating genetic lesion in retinoblastoma. The p53 gene is intact in human retinoblastoma but the pathway is believed to be suppressed by increased expression of MDM4 (MDMX) and MDM2. Here we quantify the expression of MDM4 and ...

OBJECTIVE To evaluate whether neoadjuvant intravenous chemotherapy (chemoreduction) for retinoblastoma reduces the risk for associated intracranial neuroblastic tumor (trilateral retinoblastoma). DESIGN Retrospective consecutive case series. PARTICIPANTS Two hundred fourteen consecutive children with newly diagnosed retinoblastoma treated at a major ocular oncology center from January 1, 19...

Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma. Ophthalmology. 2001;108:2106-2115. 9. Madreperla SA, Hungerford JL, Cooling RJ, Sullivan P, Gregor Z. Repair of late retinal detachment after successful treatment of retinoblastoma. Retina. 2000;20:28-32. 10. Baumal CR, Shields CL, Shields JA, Tasman WS. Surgical repair of rhegmatogenous retinal detachme...

PURPOSE To investigate the differential roles of matrix metalloproteinase (MMP)-9 and MMP-2 in the proliferation or differentiation of retinoblastoma cells. METHODS Cell proliferation assay with an MMP-9 inhibitor and cell viability assay with an MMP-2 inhibitor were performed in retinoblastoma cells with 5 ng/mL fibroblast growth factor 2 for proliferation, 0.1% bovine serum albumin for diff...

Introduction: Retinoblastoma is the most common primary ocular malignancy in pediatric age. Knudson proposed his two-hit model, allowing the distinction of retinoblastoma in two major classes: heritable and non-heritable. Retinoblastoma was first considered to arise from a well known mutation in the RB1 tumor-suppressor gene (chromosome 13q14). Currently, evidence supports that biallelic inacti...

BACKGROUND Survivors of hereditary retinoblastoma have an elevated risk of developing second malignancies, but data on the risk in middle-aged retinoblastoma survivors (ie, those with more than 40 years of follow-up) are scarce. METHODS Data from the Dutch retinoblastoma registry were used to analyze risks of second malignancies in 668 retinoblastoma survivors, diagnosed from 1945 to 2005 (me...

Retinoblastoma is a pediatric cancer of the retina most often caused by inactivation of the retinoblastoma (RB1) tumor suppressor gene. We previously showed that Rb1 loss cooperates with either co-activating the phosphatidylinositol 3-kinase (PI3K)/AKT pathway, or co-deleting Pten, to initiate retinoblastoma tumors in mice. The objectives of this study were to determine if the AKT pathway is ac...

purpose : the first report of electroretinographic changes following the intravitreal injection of carboplatin in a patient with retinoblastoma case report : recurrent tumors after chemoreduction and adjunctive local treatment in a case of familial unilateral retinoblastoma, group c, was treated with intravitreal carboplatin (10 µg) injections. results : the tumor was regressed after 3 injectio...