A 2-year-old child presents with a white reflex (leukocoria) in the right eye detected in a photo. Ultrasound shows a calcified intraocular mass. RetCam imaging and examination under anaesthesia is performed. An international classification of retinoblastoma (IIRC) is used. A group D eye has: large tumours, vitreous seeding, and subretinal seeding but an attached retina. What is the first-line management for a unilateral Group D retinoblastoma in a 2-year-old?

• A Enucleation as first-line treatment
• B Systemic neoadjuvant chemotherapy (carboplatin, vincristine, etoposide) followed by local consolidation
• C External beam radiotherapy
• D Primary intra-arterial chemotherapy (IAC) via ophthalmic artery ✓

Explanation

For unilateral Group D retinoblastoma (IIRC), intra-arterial chemotherapy (IAC) delivered selectively into the ophthalmic artery via microcatheter (Melphalan ± Topotecan) has become the first-line globe-salvaging approach at major centres, with globe salvage rates exceeding 70% for Group D eyes. Systemic chemotherapy (chemoreduction) was previously first-line but has lower salvage rates for Group D/E. Enucleation remains the choice for Group E or failed IAC. External beam radiotherapy is avoided due to secondary malignancy risk.

Reference: Khurana Comprehensive Ophthalmology, 7th ed.

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Written and medically reviewed by the StethoPrep medical team.