Management and Outcomes of Unilateral Group D Tumors in Retinoblastoma

Amin, Saima; AlJboor, Mays; Toro, Mario Damiano; Ręjdak, Robert; Nowomiejska, Katarzyna; Nazzal, Rashed Mustafa; Mohammad, Mona; Al‐Hussaini, Maysa; Khzouz, Jakub; Banat, Sara; AlJabari, Reem; Jaradat, Imad; Mehyar, Mustafa; Sultan, Iyad; Al-Nawaiseh, Ibrahim; Yousef, Yacoub A.

doi:10.2147/opth.s282741

Cited by 8 publications

(6 citation statements)

References 34 publications

(38 reference statements)

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Section: Discussionmentioning

confidence: 99%

“…There are several ways to treat retinoblastoma, and the best option depends on many parameters.There is currently a tendency toward global salvage therapy given the current breakthroughs in systemic chemotherapy, or IAC. 26 However, when we defer enucleation to salvage an eye with an active disease, especially for IIRC group D eyes, this may generate a concern about endangering the child's life in an attempt to preserve the globe and delay enucleation. 13,14 In these circumstances, further precautions should be made to recognize when therapy is ineffective and when to proceed with enucleation to avoid metastatic development and ensuing fatality.…”

Section: Discussionmentioning

confidence: 99%

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A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma

Mohammad

Shehada

Al-Nawaiseh

et al. 2023

European Journal of Ophthalmology

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Aim To compare the risk and pattern of High-Risk Pathologic Features (HRPF) in retinoblastoma between primary and secondary enucleation. Methods A retrospective analysis of 121 eyes from 118 patients who underwent enucleation at the King Hussein Cancer Center (KHCC) Amman, Jordan, between November 2009 and January 2020. Demographic information, tumor stage, time from diagnosis–to-enucleation, results of pathology, metastasis, and mortality were retrieved. Results Patients in the secondary group (49/121 eyes, 40%) were considerably younger at diagnosis (p = 0.0014), had bilateral disease (p = 0.0001), and had less-progressed disease at presentation (p = 0.016) compared to the primary enucleation. Primarily enucleated eyes were more-likely to have massive choroidal invasion (p = 0.0315) and post-laminar optic nerve invasion (p = 0.027), in spite of the finding that the overall prevalence of HRPF was similar between the two groups (35.5 percent vs. 37.5 percent; p = 0.585). The likelihood of anterior chamber invasion, was considerably higher in secondary enucleated eyes (p = 0.013). We evaluated primary and secondary enucleation for each subgroup (D and E) of the International Intraocular Retinoblastoma Classification (IIRC) and found the prevalence of HRPF was comparable (p = 0.58, 1.0, respectively). The difference in time between diagnosis-to-enucleation in secondary enucleation did not predict HRPF (p = 0.50). There was no discernible difference between primary and secondary enucleated eyes in terms of metastasis or survival (p = 0.156 and 0.44, respectively). Conclusion Systemic chemotherapy has the ability to reduce the extent of tumor expansion that has been pathologically identified. Primary and secondary enucleated eyes are comparable in low metastatic risk only when strict examination and management guidelines are followed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma

Mohammad

Shehada

Al-Nawaiseh

et al. 2023

European Journal of Ophthalmology

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“…The carboplatin, vincristine, and etoposide (CVE) protocol for chemoreduction with focal therapy continues to be the conventional first-line treatment worldwide. Amin et al [ 27 ] also reported that with systemic chemotherapy, they managed to salvage the globe in nine out of 20 eyes (45%) with group D retinoblastoma.…”

Section: Discussionmentioning

confidence: 99%

Clinicodemographic profile, management, and treatment outcomes in advanced retinoblastoma at a tertiary care center in North India

Kumar,

Sati

et al. 2023

Indian Journal of Ophthalmology

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Purpose: The study was undertaken to look into the clinicodemographic profile, management, and clinical outcomes of advanced retinoblastoma at a tertiary care center. Methods: A prospective cohort study was conducted from Jan 2019 to Dec 2022. Forty-two patients of intraocular advanced retinoblastoma were assessed. The treatment protocol was formulated based on size, extension of tumor, and laterality. Primary outcome measure was response to the treatment in terms of regression of tumor and seeds and no evidence of recurrence after 12 month in enucleated eyes. Secondary outcome measures were complications like implant exposure, metastasis, and death associated with each treatment modality. Results: The mean age of the study group was 13 months. The most common presentation was leukocoria with diminished vision. Most of the patients had group E retinoblastoma (n = 40, 95%) as per the International Classification of Retinoblastoma. In 12 patients with group E retinoblastoma, primary enucleation was performed and in six patients, secondary enucleation was done, in which initially, globe salvage treatment was tried. In 30 patients, globe salvage treatment was attempted and we could manage to save 23 eyes. The most common treatment modality was intra-arterial chemotherapy using a triple-drug regimen. One patient developed intracranial spread and died due to systemic metastasis during the follow-up period. Conclusion: The current study showed that globe salvage is possible in advanced retinoblastoma if appropriate therapy is instituted depending upon the extent of the tumor and availability of latest treatment modalities. Intra-arterial chemotherapy using triple drugs can be offered as a first-line therapy in advanced unilateral retinoblastoma as it has been found to be very effective in the present study.

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“…Early studies have reported that the salvage rates for the groups A, B, and C retinoblastoma (classified using the International Intraocular Retinoblastoma Classification, IIRC) ( Linnmurphree, 2005 ) were generally high (81–100%) compared to only about 50% for group D eyes ( Shields et al, 2006 ; Yousef et al, 2021 ; Amine et al, 2021 ). This unfavorable outcome for in group D may be attributed to the presence of massive vitreous and/or subretinal seeds.…”

Section: Introductionmentioning

confidence: 99%

Safety and Efficacy of Intravitreal Chemotherapy (Melphalan) to Treat Vitreous Seeds in Retinoblastoma

et al. 2021

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Background: Active vitreous seeds in eyes with retinoblastoma (Rb) adversely affects the treatment outcome. This study aimed to investigate the safety and efficacy of intravitreal melphalan chemotherapy (IViC) as a treatment for recurrent and refractory vitreous seeds in patients with Rb.Methods: We used a retrospective non-comparative study of patients with intraocular Rb who had vitreous seeds and were treated by IViC (20–30 μg of melphalan) using the safety-enhanced anti-reflux technique. Tumor response, ocular toxicity, demographics, clinical features, and survival were analyzed.Results: In total, 27 eyes were treated with 108 injections for recurrent (16 eyes) or refractory (11 eyes) vitreous seeds after failed systemic chemotherapy. A total of 15 (56%) were males, and 20 (74%) had bilateral disease. At diagnosis, the majority (n = 21) of the injected eyes were group D, and n = 6 were group C. Vitreous seeds showed complete regression in 21 (78%) eyes; 100% (n = 10) for eyes with focal seeds; 65% (n = 11/17 eyes) for eyes with diffuse seeds (p = 0.04); 7 (64%) eyes with refractory seeds; and 14 (87%) eyes with recurrent seeds showed complete response (p = 0.37). In total, 16 (59%) eyes developed side effects: retinal toxicity (48%), pupillary synechiae (15%), cataracts (30%), iris atrophy (7%), and retinal and optic atrophy (4%). Only one child was lost to follow-up whose family refused enucleation and none developed orbital tumor recurrence or distant metastasis.Conclusion: IViC with melphalan is effective (more for focal than diffuse seeding) and a relatively safe treatment modality for Rb that can improve the outcomes of eye salvage procedures. However, unexpected toxicity can occur even with the standard dose of 20–30 μg.

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Management and Outcomes of Unilateral Group D Tumors in Retinoblastoma

Cited by 8 publications

References 34 publications

A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma

A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma

Clinicodemographic profile, management, and treatment outcomes in advanced retinoblastoma at a tertiary care center in North India

Safety and Efficacy of Intravitreal Chemotherapy (Melphalan) to Treat Vitreous Seeds in Retinoblastoma

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