Radiotherapy Risk Management in Retinoblastoma

Risk of Secondary Malignancies

• Children with germline RB1 mutations who undergo radiotherapy have a markedly increased lifetime risk of secondary malignancies, especially sarcomas and melanomas. 1, 2
• Approximately 45 % of retinoblastoma cases are hereditary, involving germline RB1 mutations, which heightens radiation‑related risk considerations. 1
• The irradiated field remains a persistent high‑risk zone for secondary tumor development throughout the patient’s life. 1

Importance of Genetic Testing

• Immediate determination of germline RB1 status is essential because it fundamentally alters the risk‑benefit calculus for radiotherapy in retinoblastoma. 1, 3
• Delaying genetic testing is discouraged; knowing germline RB1 status is required to properly counsel families about radiation‑associated risks. 1, 3

Decision‑Making Factors for Radiotherapy

• Assessment of tumor size and location is a key factor when considering radiotherapy after chemotherapy failure. 2
• Germline mutation status must be incorporated into the evaluation of radiotherapy suitability. 2

Long‑Term Surveillance

• All patients who receive radiotherapy for retinoblastoma require lifelong monitoring for secondary malignancies. 1