Orbital Retinoblastoma: From Palliation to Cure by Sequential Multimodal Multidisciplinary Therapy - A Case Report

Orbital retinoblastoma (RB) is a form of advanced-stage RB and typically carries poor prognosis, with mortality rates ranging from 25% to 100% [Br J Ophthalmol. 1990;74(2):97-8]. While new multimodal treatment protocols have been proposed, there remains a paucity of reported successful treatment outcomes before standard treatment protocols can be established. In many developing nations, orbital RB is frequently palliated due to presumed poor treatment outcomes.

We highlight a case of a 6-year-old child who presented with a unilateral fungating orbital RB without regional or systemic spread. After 4 cycles of neoadjuvant chemotherapy, there was complete clinical regression of the tumour to within the globe. Following an ocular enucleation with histopathologic control, he underwent placement of a primary orbital implant followed by adjuvant proton beam therapy (PBT) to the orbit and remains well till this day.

This case underscores the importance of multimodal combination therapy in improving survival outcomes, highlighting the promising impact of neoadjuvant systemic chemotherapy in reducing tumour burden even in advanced RB. It also draws attention to the use of PBT as a superior alternative to conventional external beam radiotherapy, due to its ability to reduce dose splash to surrounding organs, reducing the chance of secondary malignancies [Indian J Ophthalmol. 2024;72(6):778-88]. Above all, this case challenges the historical prognosis of orbital RB, demonstrating that with a tailored and comprehensive approach, even children with advanced disease can attain disease-free survival. It serves as a call to action for the clinical community to continue pursuing evidence-based treatment protocols that can transform advanced RB care.