Intra-arterial chemotherapy in refractory and advanced intraocular retinoblastoma

Abstract

Purpose: To evaluate the efficacy of secondary and salvage intra-arterial chemotherapy (IAC) as a globe salvage treatment modality in advanced and refractory intraocular retinoblastoma.

Methods: A retrospective chart review of advanced intraocular retinoblastoma (groups D and E International Classification of Retinoblastoma [ICRB] classification) patients refractory to intravenous chemotherapy (IVC) and undergoing IAC as the secondary and salvage treatment modality between December 2018 and June 2021 was carried out. All patients underwent the IAC procedure by super-selective ophthalmic artery catheterization and with triple-drug chemotherapeutic agents of melphalan, topotecan, and carboplatin. Data were collected about tumor regression, eye salvage, metastasis, and survival outcome at follow-up.

Results: Out of 13 patients, 12 patients received secondary IAC after being primarily treated with IVC and focal therapies and one patient received rescue IAC after recurrence following primary IAC. Mean number of IAC cycles administered was 2. Overall, globe salvage rate was 53.84%, with a mean follow-up of 17.53 months (range 6-37 months), three patients had enucleation for residual tumor or tumor recurrence. One patient developed metastasis post enucleation and two patients who were lost to follow-up after enucleation advice for residual tumor developed orbital tumor extension and eventually died of metastasis.

Conclusion: Secondary triple-drug IAC following failure of IVC, along with other adjunct treatment modalities might a be a cost-effective option for eye salvage in advanced intraocular retinoblastoma patients who refuse enucleation, with a globe salvage rate of 53.84%. It can also be an effective approach to improve treatment compliance and can help in addressing the barrier of treatment refusal when enucleation is advised.

Keywords: Globe salvage; intra-arterial chemotherapy; retinoblastoma.

Figure 1

Unilateral group E RB in a child presenting with retinal detachment (a) and tumor filling the vitreous cavity on B scan (b). Appearance after IVC showing residual active retinal tumor and subretinal seeds and vitreous seeds (c). Appearance after triple-drug IAC and add-on focal and IVitC at 2-year follow-up showing complete tumor regression (d). Massive retinal tumor recurrence with dispersed vitreous hemorrhage in the left eye of a bilateral RB child post fellow eye enucleation and IVC (e and f). Appearance after the first cycle of triple-drug IAC (g) and appearance after four cycles of IAC and add-on focal therapy at 9-month follow-up showing complete tumor regression and disk pallor (h). IAC = intra-arterial chemotherapy, IVC = intravenous chemotherapy, IVitC = intravitreal chemotherapy, RB = retinoblastoma

Figure 2

Group D RB in the worse eye of a child with bilateral RB showing diffuse vitreous seeds and solid retinal tumor and diffuse subretinal seeds (a and b). Appearance after IVC showing partial tumor regression and residual active vitreous seeds and subretinal seeds (c and d). Complete regression of vitreous seeds and retinal tumor after one cycle of triple-drug IAC followed by IVitC and cryotherapy (e and f). IAC = intra-arterial chemotherapy, IVC = intravenous chemotherapy, IVitC = intravitreal chemotherapy, RB = retinoblastoma

Figure 3

Unilateral Group E RB with tumor filling the vitreous cavity (a). Appearance after six cycles of IVC showing partial tumor regression (b). Appearance at 10 months follow-up after IAC and plaque brachytherapy showing complete tumor regression (c). Group D RB in a child with bilateral RB with fellow eye enucleated for Group E RB (d). Appearance after six cycles of IVC showing partial regression of tumor with residual active retinal tumor and vitreous seeds (e). Complete tumor regression at 1 year follow-up after one cycle of triple-drug IAC followed by plaque brachytherapy, IVitC, and focal therapy (f). IAC = intra-arterial chemotherapy, IVC = intravenous chemotherapy, IVitC = intravitreal chemotherapy, RB = retinoblastoma

Figure 4

Unilateral Group D RB in a 9-year-old child presenting with diffuse vitreous seeds, subretinal seeds, and subretinal fluid (a). Appearance after six cycles of IVC and three cycles of triple-drug IAC showing good response with regression of subretinal fluid and minimal residual active tumor at the periphery (b). Patient was continued on focal therapy and at 2 months follow-up, developed total rhegmatogenous retinal detachment with residual active subretinal seeds and underwent enucleation for the same (c). IAC = intra-arterial chemotherapy, IVC = intravenous chemotherapy, RB = retinoblastoma