A 2-year-old child is brought with leukocoria. Fundus under EUA shows a white mass with calcification. Retinoblastoma is suspected. Genetic testing reveals a germline RB1 mutation. The tumour in the right eye is confined to the retina with no optic nerve or choroidal invasion, classified as group C (IIRC classification). The contralateral eye is normal. What is the most appropriate management?
- A Immediate bilateral enucleation to prevent metastasis
- B External beam radiotherapy as primary treatment
- C Intra-arterial chemotherapy (ophthalmic artery chemosurgery) to salvage the eye ✓
- D Intravitreal melphalan injection as sole treatment
Explanation
Intra-arterial chemotherapy (IAC) — superselective ophthalmic artery infusion of melphalan — is now the preferred globe-salvaging approach for group C/D unilateral retinoblastoma, achieving high tumour control with preservation of vision and the globe. Bilateral enucleation is inappropriate when the fellow eye is healthy and globe salvage is possible in a group C eye. External beam radiotherapy risks secondary malignancies (particularly osteosarcoma within the field), especially critical in germline RB1 mutation carriers; it is now reserved for orbital disease. Intravitreal melphalan alone is used for vitreous seeds but not as sole therapy for a solid group C tumour.
Reference: Khurana Comprehensive Ophthalmology, 7th ed.
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Written and medically reviewed by the StethoPrep medical team.