Nokea
A 3-year-old boy is referred to you for evaluation of right leukocoria. Funduscopic examination under anesthesia reveals a large amelanotic mass occupying more than two-thirds of the vitreous space in his right eye, with massive retinal detachment, consistent with group E retinoblastoma. The left eye is normal. An MRI confirms the funduscopic findings and shows no extraocular disease. What is the most appropriate next step in the management of this child's disease?
Correct Answer: A
Rationale: The most appropriate next step in the management of the child's retinoblastoma is enucleation (choice A). Enucleation, the surgical removal of the affected eye, is indicated when there is a large intraocular tumor with extensive retinal detachment, as in this case. Enucleation can provide local control of the disease and prevent systemic spread. It is considered the standard treatment for advanced retinoblastoma to prevent metastasis.
Systemic chemotherapy (choice B) is not the first-line treatment for advanced retinoblastoma with massive intraocular involvement. Brachytherapy (choice C) involves the placement of a radioactive source near the tumor, which may not be effective in this case of extensive intraocular disease. Needle biopsy (choice D) is not recommended as the primary management strategy due to the risk of seeding tumor cells outside the eye. Therefore, enucleation is the most appropriate next step in this scenario.