Secondary Craniofacial Sarcomas Following Retinoblastoma: A Systematic Review.

OBJECTIVE

We conducted the largest systematic review of individual patient data to characterize secondary craniofacial sarcomas following retinoblastoma.

METHODS

We conducted a systemic search of the PubMed databases and compiled a comprehensive literature review. Student t tests were used to evaluate differences between variables. Kaplan-Meier analysis was used to estimate survival. Statistical significance was assessed using a log-rank test.

RESULTS

We analyzed 220 cases of secondary craniofacial sarcomas, including 112 osteosarcomas. The average age (±SD) of onset for retinoblastoma was 1.20 ± 2.77 years. External-beam radiotherapy was delivered in 207 patients (94.0%) and chemotherapy was delivered in 53 patients (24.0%) patients. The latency period between retinoblastoma diagnosis and the onset of secondary sarcomas was 12 years. Cranial extension was found in 66 patients (30.0%). The median overall survival was worse with cranial extension (P = 0.0073). In cranial extended patients, the median survival in patients who received chemotherapy was 41 months, whereas patients who did not receive chemotherapy had a median survival of 12 months (P = 0.0020).

CONCLUSIONS

The risk of incidence of secondary sarcomas in retinoblastoma patients warrants longer follow-up periods. Moreover, chemotherapy should be considered as a potential treatment option for secondary cranial sarcomas following retinoblastoma.