Intraocular retinoblastoma features and treatment outcomes according to patient age at initial presentation.

CLINICAL RELEVANCE: Age at presentation is a key biological and clinical determinant in intraocular retinoblastoma, informing tailored therapeutic approaches that balance tumour control, vision preservation, and minimisation of long-term treatment morbidity.

BACKGROUND

Clinical features and long-term treatment outcomes according to age at presentation in retinoblastoma patients treated with frontline intravenous chemotherapy have been reported from developed countries only.

METHODS

The records of 185 retinoblastoma patients (94 bilateral and 91 unilateral) treated with frontline intravenous chemotherapy (6 courses of vincristine, etoposide, and carboplatin) between October 1998 and June 2024 were retrospectively reviewed.

RESULTS

Primary enucleation was performed in one eye of 18 bilateral cases. There were 102 (149 eyes), 36 (56 eyes), 17 (22 eyes), 16 (18 eyes), and 14 (16 eyes) patients in the ≤12 months, >12-≤24 months, >24-≤36 months, >36-≤48 months, and >48 months age groups, respectively. Of the 21 patients with a positive family history, 19 were in the ≤12-month age group, indicating a significant association with younger age at presentation (p = 0.016). The rate of advanced group (D/E) tumour detection was higher in patients > 36 months compared to patients ≤ 36  months old (55.9% [19/34] vs. 33.9% [77/227], p = 0.005). The frequency of vitreous seed detection was significantly higher in >24 months old vs ≤ 24 months old  patients (57.1% vs 29.8%, p = 0.001).    With increasing patient age, intravitreal chemotherapy (p = 0.001) and external beam radiotherapy (p = 0.029) were required more frequently. The 6-year globe salvage rate was significantly higher in ≤12 months age group (p < 0.001).

CONCLUSION

Retinoblastoma patients with a family history are diagnosed at earlier ages. The incidence of group D and E retinoblastoma increases in older ages. Starting chemoreduction at an early age allows for better tumour control and increases eye preservation rates. No deaths were observed among the patients included in the study.