Chemotherapy in Retinoblastoma: Current Approaches

Area of Science:

• Ophthalmology
• Pediatric Oncology
• Medical Oncology

Background:

• Retinoblastoma (RB) is the most frequent malignant intraocular tumor in children.
• Historically, enucleation and radiotherapy were standard; chemotherapy is now the preferred eye-sparing approach.

Approach:

• Chemotherapy administration routes include systemic, subconjunctival, intra-arterial, and intravitreal.
• Systemic therapy aims for tumor size reduction (chemoreduction) or metastasis prevention (adjuvant therapy).
• Super-selective intra-arterial infusion offers targeted delivery, minimizing systemic side effects, and can be used for primary or recurrent tumors.

Key Points:

• Intravitreal chemotherapy addresses persistent vitreous seeding.
• Extraocular RB involves orbital invasion or metastasis, with Central Nervous System (CNS) involvement carrying a grave prognosis.
• Treatment for advanced disease may involve high-dose salvage chemotherapy with stem cell rescue and radiotherapy.

Conclusions:

• Chemotherapy represents a cornerstone in managing intraocular and extraocular retinoblastoma.
• Diverse administration routes allow for tailored treatment strategies, optimizing efficacy and minimizing toxicity.
• Advances in chemotherapy and supportive care are crucial for improving outcomes in advanced or metastatic retinoblastoma.