Rescue intra-arterial chemotherapy following retinoblastoma recurrence after initial intra-arterial chemotherapy.

OBJECTIVE

To determine the efficacy of rescue intra-arterial chemotherapy (IAC) for retinoblastoma recurrence following failed initial IAC.

METHODS

Retrospective, non-comparative, interventional case series of 12 eyes in 12 patients.

METHODS

Rescue IAC employed chemotherapy agents of melphalan (5mg, 7.5mg) alone or with additional topotecan (1mg).

METHODS

Tumor control and globe salvage.

RESULTS

The median patient age at initial presentation was 16 months. At initial examination, the International Classification of Retinoblastoma grouping was group B (n=1), group D (n=7), or group E (n=4). The initial IAC was primary in 5 cases (42%) and secondary following failure of intravenous chemotherapy in 7 (58%). In all cases, initial IAC was delivered using melphalan 3mg (n=3), melphalan 5mg (n=7), or combination melphalan 5mg/topotecan 1mg (n=2) for a median of 3 cycles. The mean interval from initial IAC to recurrence necessitating rescue IAC was 5 months (median 4, range 2-10 months). Of the 12 patients, 3 (25%) had undergone previous enucleation of the opposite eye and the rescue IAC was planned for the only remaining eye. Rescue IAC was delivered for recurrent solid tumor (n=1), recurrent subretinal seeds (n=7), recurrent vitreous seeds (n=1), or combination recurrent subretinal/vitreous seeds (n=3). IAC was technically successful through the ophthalmic artery in 9 cases (75%) or the middle meningeal artery in 3 (25%). Rescue IAC involved median 3 cycles (mean 3, range 2-4 cycles) of higher dose melphalan in 4 cases (33%) or combination melphalan/topotecan in 8 (67%). At mean follow-up of 20 months (median 14 months, range 7-36 months), complete tumor control was achieved in 9 eyes (75%) and globe salvage in 8 eyes (67%). Of the 3 failure eyes, all were initially groups D or E, previously treated with initial IAC, and 2 had previous intravenous chemotherapy. There were 4 eyes that came to enucleation for persistent subretinal/vitreous seeds (n=3) or neovascular glaucoma without viable tumor (n=1). There was no case of cerebrovascular stroke, systemic metastasis, or death.

CONCLUSIONS

Rescue IAC following retinoblastoma recurrence after initial IAC provided tumor control in 75% of cases and globe salvage in 67%. Rescue IAC can be considered in children who fail initial IAC, especially if the opposite eye has been enucleated.