High-Risk Retinoblastoma Based on International Classification of Retinoblastoma: Analysis of 519 Enucleated Eyes

doi:10.1016/j.ophtha.2012.10.044

Ophthalmology

Volume 120, Issue 5, May 2013, Pages 997-1003

https://doi.org/10.1016/j.ophtha.2012.10.044 Get rights and content

Purpose

To determine the correlation between the International Classification of Retinoblastoma (ICRB) and histopathologic high-risk retinoblastoma.

Design

Retrospective study.

Participants

A total of 519 patients.

Intervention

Primary enucleation.

Main Outcome Measures

High-risk retinoblastoma, metastasis, and death.

Results

Of 519 primarily enucleated eyes, 87 (17%) were classified as group D and 432 (83%) were classified as group E on the basis of the ICRB. High-risk retinoblastoma was identified in 23% (117/519) of enucleated eyes, including 17% (15/87) group D and 24% (102/432) group E eyes. High-risk histopathologic features of retinoblastoma included anterior chamber involvement (5/15 [33%] group D eyes, 31/102 [30%] group E eyes), isolated massive posterior uveal invasion ≥3 mm (7/15 [47%] group D eyes, 22/102 [22%] group E eyes), isolated post-laminar optic nerve invasion (2/15 [13%] group D eyes, 46/102 [45%] group E eyes), and any combination of posterior uveal invasion and optic nerve involvement (7/15 [47%] group D eyes, 37/102 [36%] group E eyes). On logistic regression analysis, massive posterior uveal invasion ≥3 mm was more common in group D eyes (P = 0.0442), and post-laminar optic nerve invasion was more common in group E eyes (P = 0.0390). Of 117 patients with high-risk retinoblastoma, systemic adjuvant chemotherapy was administered in 83 patients (71%). Systemic metastasis developed in 0% (0/15) of those with high-risk group D retinoblastoma and 10% (10/102) of those with high-risk group E retinoblastoma over a mean follow-up period of 78 months (median, 62 months; range, 1–419 months). There was no metastasis in any patient (n = 402) classified with non–high-risk retinoblastoma. Of the 10 patients who developed metastasis, 4 had received prior adjuvant chemotherapy and 6 had no prior adjuvant chemotherapy. There was no metastasis in high-risk patients treated with vincristine sulphate, etoposide phosphate, and carboplatin (VEC). Death from metastasis occurred in 4% of high-risk patients (5/117).

Conclusions

On the basis of the ICRB, 17% of group D and 24% of group E eyes are at increased risk for metastatic disease. In this study, 8% of patients developed metastasis. There was no metastasis in any patient classified with non–high-risk retinoblastoma.

Financial Disclosure(s)

The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Section snippets

Materials and Methods

This was a retrospective, nonrandomized, interventional case series. Institutional review board approval was obtained. The medical records of all patients with retinoblastoma managed with enucleation on the Ocular Oncology Service at Wills Eye Institute in Philadelphia between January 1, 1975, and December 15, 2011, were reviewed. Patients who underwent primary enucleation for treatment of retinoblastoma were included in this study. Those who underwent secondary enucleation after failure of

Results

Of 639 eyes enucleated for retinoblastoma during this time period, 519 (81%) underwent primary enucleation and were included in this study. Secondary enucleation was performed in 120 eyes (19%), and these were excluded from the study.

Of the 519 primarily enucleated eyes, 87 (17%) were classified as group D and 432 (83%) were classified as group E on the basis of the ICRB. There were no eyes in groups A, B, or C that underwent primary enucleation. According to RE classification, there were 2

Discussion

High-risk retinoblastoma detected on histopathologic examination of enucleated eyes can identify children at risk for metastatic disease. High-risk features include tumor invasion into the anterior chamber, an area of posterior uveal invasion ≥3 mm, post-laminar optic nerve invasion, or a combination of any nonmassive posterior uveal invasion (<3 mm) with any degree of nonretrolaminar optic nerve invasion.¹¹

Wilson and associates¹³ explored the relationship of ICRB classification with high-risk

Acknowledgment

Statistical analysis was provided by Rishita Nutheti, PhD, Hyderabad, India.

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All retinoblastoma-related deaths occurred in patients with both massive choroidal invasion and PLONI. These findings could not establish that massive choroidal invasion is an independent risk factor for poor outcome.
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Microscopic foci of choroidal invasion without viable tumor cells were also observed. However, high-risk histopathological features for metastasis,13,14 such as anterior structure, massive choroidal, and optic nerve invasions and scleral infiltration, were not observed. Follow-up brain magnetic resonance imaging was performed regularly for screening and monitoring the optic nerve invasion.
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Retinoblastoma is a curable cancer when management is early and prompt.
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A retrospective study was carried out from January 2014 to December 2018 (5 years) at the department of ophthalmology in Campus-University Teaching Hospital, Lomé, Togo. It focused on children with retinoblastoma. It included patients with diagnosis supported by ocular ultrasonography, orbito-cerebral computed tomography scan and/or histopathological examination.
Among the 75 children with ocular cancer, 26 (34.7%) were diagnosed with retinoblastoma. The mean age at presentation was 31 months. The mean time from symptom onset to diagnosis was 15 months. A predominance of proptosis (65.4%), unilateral involvement (76.9%), extraocular tumor extension (65.4%) and endophytic form (96.15%) was found. Of the 32 eyes with retinoblastoma, 7 (21.9%) were enucleated after a mean time from diagnosis to enucleation of 6 months. Two cases of complete remission, 3 cases of tumor recurrence, and 2 cases lost to follow-up were noted.
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: Manuscript no. 2012-658.

: Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

: Support was provided by Carlos G. Bianciotto Retinoblastoma Research Fund c/o Eye Tumor Research Foundation, Philadelphia, PA (C.L.S., J.A.S.); The Lucille Wiedman Fund for Pediatric Eye Cancer, Philadelphia, PA (C.L.S., J.A.S.); Lift for a Cure, Morrisdale, PA (C.L.S.); and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Institute (R.C.E.). The funders had no role in the design and conduct of the study; in the collection, analysis, and interpretation of the data; and in the preparation, review, or approval of the manuscript. Dr. Shields has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

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Original articleHigh-Risk Retinoblastoma Based on International Classification of Retinoblastoma: Analysis of 519 Enucleated Eyes

Purpose

Design

Participants

Intervention

Main Outcome Measures

Results

Conclusions

Financial Disclosure(s)

Section snippets

Materials and Methods

Results

Discussion

Acknowledgment

Int J Radiat Oncol Biol Phys

Ophthalmology

Ophthalmology

Ophthalmology

Retinoblastoma

Staging and treatment strategies

Intra-arterial chemotherapy for retinoblastoma: report no. 1, control of retinal tumors, subretinal seeds, and vitreous seeds

Arch Ophthalmol

Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy

Curr Opin Ophthalmol

Postenucleation adjuvant therapy in high-risk retinoblastoma

Arch Ophthalmol

Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma

Arch Ophthalmol

Intra-arterial chemotherapy for retinoblastoma: report no. 2, treatment complications

Arch Ophthalmol

Original article
High-Risk Retinoblastoma Based on International Classification of Retinoblastoma: Analysis of 519 Enucleated Eyes