Retinoblastom: Vorschlag eines multimodalen Behandlungskonzeptes für Patienten mit intraokularem Retinoblastom in Österreich

Abstract

Retinoblastoma is the most common intraocular malignancy in childhood. If confined to the globe and managed with current treatment strategies, more than 90% of children survive with preservation of vision in at least one eye, even in bilateral retinoblastoma. Enucleation of the involved eye in unilateral retinoblastoma and of the more involved eye in bilateral disease, together with external beam radiotherapy in advanced bilateral retinoblastoma, formed the two cornerstones of treatment for many years and led to an increase in survival to over 90%. In the early 1990s the extent of the risk of second cancers in the field of radiation became known, the risk increasing by 10% per decade of life. As a consequence, chemotherapy-based regimens were developed as alternative treatments. During the past ten years retinoblastoma treatment has fundamentally changed, with a trend away from enucleation and external beam radiotherapy towards conservative treatments aiming at preservation of the affected globe(s) in selected patients. Systemic neoadjuvant chemotherapy induces tumor regression (chemoreduction), and residual regressed tumor is then treated focally with, for example, transpupillary thermotherapy, cryotherapy and plaque radiotherapy (consolidation). Between 1984 and 2004, 27 patients were treated at the department of ophthalmology in collaboration with the department of pediatrics at the Medical University of Graz. Before 2001, the affected eyes of all patients with unilateral retinoblastoma were enucleated (10 of 10), as were 6 of 7 of the more involved eyes of patients with bilateral disease. A globe-sparing strategy was introduced in 2001 and since then eligible patients have been treated with chemoreduction and focal therapy; 2 of 5 eyes with unilateral disease were salvaged, and both eyes of a patient with bilateral disease. We discuss current treatment options and present a proposal for the management of intraocular retinoblastoma in children in Austria, the Austrian retinoblastoma study, RB A-2003.