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Introduction: Laser therapy is a cornerstone for control of intraocular retinoblastoma, after chemotherapy has brought the disease under initial control. Since first described over 6 decades ago, l...

To evaluate our experience with systemic Toptecan (TPT) chemotherapy as a second-line systemic chemotherapeutic regimen for treatment of refractory or recurrent intraocular retinoblastoma (RB).A retrospective case series of 14 eyes from patients with intraocular RB who received systemic TPT as second-line chemotherapy from April 2008 until June 2010. The following data were collected: patient demographics, laterality, international intraocular retinoblastoma stage (ICRB) at diagnosis, treatment received before and after TPT, side effects related to TPT, eye salvage, and survival.The median age at diagnosis was 5 months (range, 1-16 months), and the median age at starting TPT was 10 months (range, 8-24 months). There were 6 (60%) females and 9 (90%) patients; all with bilateral retinoblastoma. The median number of TPT cycles was three per patient (range, 1-6), and the total number of administered cycles was 29. After TPT therapy; 4 (29%) eyes showed favorable response, 3 (21%) eyes showed minimal regression, 5 (36%) eyes had stable disease, and 2 (14%) eyes showed tumor progression. At a median follow-up of 48 months; 9 (64%) eyes were salvaged, 3 (21%) eyes received radiation therapy, and 3 (21%) eyes were enucleated (one was post radiation). Grade 3/4 neutropenia were noticed in a total of 59% of given cycles and admission for febrile neutropenia was required after seven cycles.Our report suggests that systemic TPT chemotherapy could be used as a salvage second-line regimen with low toxicity for patients with progressive intraocular retinoblastoma if systemic therapy is needed.

Introduction: Survival for patients with intraocular retinoblastoma (RB) is excellent. Over the past two decades, the focus of therapy has shifted to emphasize ocular salvage and vision preservation. The combination of chemoreduction and aggressive focal therapies has formed the new standard approach to most patients with advanced intraocular RB. However, seeding of the vitreous cavity continues to be the primary reason for treatment failure in these patients.Areas covered: Local delivery methods, including periocular injections, intra-ophthalmic artery infusions, and intravitreal injections, have been developed in the hope of controlling disease in advanced cases. This review highlights the current approach to therapy for patients and discusses these new local delivery techniques and the discovery of novel therapeutics that may help to further improve ocular outcomes for patients with intraocular RB.Expert opinion: Due to the rarity of this childhood disease, clinical efforts to improve ocular salvage an...

Although the survival rates of retinoblastoma patients in the USA and other developed nations approach 98% and globe salvage rates have increased dramatically over time, many challenges still face both the ocular and pediatric oncologists who care for the patients with this disease. In this review, we discuss the current strategy for the utilization of chemotherapeutic agents for retinoblastoma, including intravenous delivery, subconjunctival injections and intra-arterial administration. We also review the current uses of chemotherapy for metastatic disease. The challenges and complications encountered with chemotherapeutic treatment strategies in this unique patient population will be discussed. Finally, we address the novel pharmacotherapies and delivery systems currently being explored in the laboratory that will soon be critical in clinical settings.

One of the treatment options for intraocular retinoblastoma is thermochemotherapy with diode laser combined with chemotherapy. Very little is known about the evolution of laser scars resulting from diode laser treatment for retinoblastoma. We report a case of atrophic chorioretinal scar and focal scleral bowing after thermochemotherapy with diode laser for retinoblastoma.

Results from experimental tumours suggest that the mechanism of action of photodynamic therapy (PDT) involves both a direct killing of tumour cells, and a secondary effect− resulting from vascular damage. We have investigated the possible vascular changes induced by PDT in an intraocular retinoblastoma-like rat tumour model using the 86RbCl extraction procedure. Light irradiation (90 J/cm2; 633 nm; 30 min) of intraocular tumours 24 h after an intraperitoneal injection of 5 mg/kg Photofrin II produced an increase in the tumour uptake of 86RbCl during the treatment period. However, 24 h later these values had decreased to 25 % of that normally found in control animals. these effects were observed in both the tumour material and associated normal eye tissue, but not in PDT treated normal eyes without tumours. the results confirm that the vasculature of this eye tumour model is a target for some of the PDT effects.

The influence of the very limited potential for an increase of blood supply to the inner eye on the growth of intraocular retinoblastoma are listed, demonstrated, and discussed. It is typical for the most malignant retinoblastomas to present with the most massive ischemia and necrosis. The limited possibilities for vascular enlargement and neovascularization lead to cuff-like perivascular survival of neoplastic cells with necrosis in the interspaces. Indirect supply from retinal and ciliary sources explain layer-like neoplastic growth on surface and base of tumors as well as separation of living neoplastic cells, attraction towards exit areas, cyst formation, and seeding. These processes may contribute to extraocular extension. Recognition of the limits of intraocular nutrition also is important for prognosis and therapy in cases of retinoblastoma.