Systemic chemotherapy as a new conservative treatment for intraocular retinoblastoma

Abstract

Retinoblastoma is the most common malignant intraocular tumor in childhood. With advances in the methods for early detection of this disease, the survival rate is over 90% in developed countries. The management of intraocular retinoblastoma has gradually changed over the past few decades. Every effort has been made to save life, with the preservation of the eye and sight, if possible. External beam radiotherapy has been a standard treatment for medium and large, or visually threatening, intraocular retinoblastoma, but it markedly increases the risk of cosmetic deformities and secondary cancer in children with germline RB mutations. For the past decade, primary systemic chemotherapy called "chemoreduction" has been employed to avoid radiotherapy and enucleation. This article gives an overview of the results of current trials of primary chemoreduction for intraocular retinoblastoma, and discusses its role and its limitations in conservative treatment. The article also discusses future directions to expand the indications for this treatment. Many children with advanced intraocular retinoblastoma could be spared external beam radiotherapy and enucleation, mostly as a result of chemoreduction and focal methods. Chemoreduction combined with focal treatments will continue to play an important role in the conservative management of children with intraocular retinoblastoma, possibly even in children with advanced disease, with the combined use of multidrug-resistance modulators.

Quality of life of adult retinoblastoma survivors in the Netherlands

“…However, it is estimated that approximately 10% of patients with unilateral RB still have a germline mutation [2]. The aim of the treatment of RB is to cure the disease and preserve vision [3]. In the western world RB has an excellent 5-year survival rate of more than 90% [4].…”

Beta-lapachone inhibits proliferation and induces apoptosis in retinoblastoma cell lines

“…An overall survival rate between 90 and 95% can be achieved with combinations of chemotherapy, external beam radiotherapy, plaque radiotherapy, laser photocoagulation, cryotherapy, and enucleation. 3 In the developing world, however, RB remains a frequently fatal disease.…”

Effect of Curcumin on Lung Resistance-Related Protein (LRP) in Retinoblastoma Cells

“…Thus, alternative drugs are needed as chemosensitizers. 8 Extensive research has been made to discover new drugs using plant extracts. These phytochemicals have the advantage of being dietary components that are less toxic to animals and inexpensive.…”

“…6 Cyclosporine A (CsA) is the chemosensitizer used along with the chemotherapy protocol in the management of retinoblastoma. 8 It has been shown to reverse P-gp-mediated MDR by increasing the intracellular concentrations of chemotherapeutic 846 S. Thiyagarajan et al…”