Histopathologic risk factors in retinoblastoma: A retrospective study of 172 patients treated in a single institution

Khelfaoui, Fatima; Validire, Pierre; Aupérin, Anne; Quintana, E.; Michon, Jean; Pacquement, Hélène; Desjardins, L.; Asselain, B; Schlienger, P; Vielh, Philippe; Jl, Dufier; Zucker, J M; Doz, François

doi:10.1016/s0002-9394(14)71991-9

Cited by 24 publications

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“…[26][27][28][29][30][31][32] Seventy-five percent of enucleated eyes in our series had one or more HHRF for metastasis. Optic nerve invasion was the commonest site of extraocular invasion in our series and correlates with that in other studies.…”

Section: Discussionmentioning

confidence: 99%

“…10 Our study has added to the importance of subjecting all enucleated eyes for histopathological analysis in order to identify those with HHRFs, the presence of which have adverse predictive value on prognosis. [26][27][28][29][30][31] Progressively small numbers of patients continued with follow up thus for 12 and 18 months durations there were only 1 without metastasis and 1 with metastasis respectively. This was also reflected in more than a third of the patients either abandoning treatment, 34.8%, or discharged with advanced disease and financial difficulty (8.7%), the latter group for palliative care at peripheral hospitals near their homes.…”

Section: Discussionmentioning

confidence: 99%

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Presentation of retinoblastoma at a paediatric eye clinic in Ghana

Essuman

Ct²,

Akafo³

et al. 2011

Ghana Medical Journal

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SUMMARYBackground: Retinobalstoma, the commonest childhood malignant intraocular tumour, is usually diagnosed early with over 90% survival rate in developed countries. In developing countries, the diagnosis is late resulting in less than 50% survival. Objective: To determine retinoblastoma stages at presentation and patients' outcomes. Design: Retrospective case series. Methods:The clinical and histopathological records of children with retinoblastoma seen from May 2004 to December 2005 were studied. Data was analysed for mode of presentation, laterality, clinical staging using Reese-Ellsworth (R-E) classification, histopathological high risk features (HHRF) for metastasis, and patient outcome. Setting: Ophthalmology Unit, Korle-Bu Teaching Hospital, Ghana. Results: Twenty-three patients presented with retinoblastoma over the period. Males were 12(52.2%). The age range from 1 to 84 months, mean 36.3(22.15) and median (36 months). Nineteen (82.6%) had unilateral and 4(17.4%) bilateral disease. The common clinical presentations were leukocoria in 20(87.0%), proptosis 8(34.8%), strabismus 5(21.7%) and red eye 5(21.7%). The clinical features were commensurate with R-E stage V in 20(87.0%) patients, 2(8.7%) with orbital recurrence and 1(4.3%) with post-enucleation anophthalmos. HHRFs were present in 9(75%) enucleated eyes with invasion of optic nerve as the commonest site (7/9). The patients were followed up for 1 day to 19 months. Eight abandoned treatment, 2 were discharged for palliative treatment, 2 out of 5 with metastasis died and 6 had no metastases at their last visit. Common sites for metastasis were the bone marrow, brain and orbit. Conclusion: Majority of the patients presented with advanced disease manifesting as leukocoria, proptosis, RE stages V disease and poor outcome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Presentation of retinoblastoma at a paediatric eye clinic in Ghana

Essuman

Ct²,

Akafo³

et al. 2011

Ghana Medical Journal

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“…12,16,17 Following enucleation, the incidence of high-risk histopathologic features has varied from 7% to 9% for anterior segment invasion, 8,17 12% to 42% for choroidal invasion, 5,7-9,17 8% to 12% for scleral invasion, 5,8,9,17 2% to 20% for extrascleral invasion, 5,8,9,17 6% to 28% for invasion of postlaminar optic nerve, 5,6,8,9,17,18 1% to 38% for involvement of optic nerve to surgical transection. 5,6,7,8,9,17,18 In a recent comprehensive report on histopathologic findings following enucleation in 297 According to Chantada and associates, 12 there are world disparities in risk definition and management of RB. On the basis of our previous experience, we believe that anterior segment invasion, massive posterior uveal invasion of 3 mm or greater, postlaminar optic nerve invasion, or a combination of any degree of posterior uveal and optic nerve invasion pose a risk; therefore, we include these 4 factors in our definition as high-risk.…”

Section: Discussionmentioning

confidence: 99%

“…1,4,5 In RB, there are histopathologic factors that have been identified from enucleated eyes predictive of the development of metastatic disease and related mortality. [6][7][8][9] Patients demonstrating such risk factors are often given postenucleation adjuvant chemotherapy for protection from metastasis and improvement in survival. 10 There is some controversy regarding the exact definition of high-risk histopathologic features 11,12 and further debate regarding the most effective chemotherapeutic protocol for treatment of such patients.…”

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confidence: 99%

Postenucleation Adjuvant Chemotherapy With Vincristine, Etoposide, and Carboplatin for the Treatment of High-Risk Retinoblastoma

Kaliki

Shields²,

Shah³

et al. 2011

Arch Ophthalmol

111

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Background: Analysis of 52 eyes with high-risk retinoblastoma managed with postenucleation adjuvant chemotherapy using vincristine sulfate, etoposide phosphate, and carboplatin showed no evidence of systemic metastasis in any case during a mean (range) follow-up of 66 (12-202) months.Purpose: To determine the efficacy of postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin in the prevention of metastasis for patients with high-risk retinoblastoma.Methods: Retrospective, nonrandomized, interventional case series of 52 eyes in 51 patients with high-risk retinoblastoma consisting of tumor invasion into the anterior segment, posterior uvea 3 mm or greater, postlaminar optic nerve, or any combination of posterior uvea and optic nerve involvement.Results: Of 51 consecutive patients with high-risk retinoblastoma, there were 30 males (59%) and 21 females (41%), with a median age of 28 months at diagnosis. All 52 eyes were classified as group E. The main histopathologic risk factors included anterior segment invasion ( 7[13%]), isolated massive posterior uveal invasion of 3 mm or greater (6 [12%]), isolated postlaminar optic nerve invasion (15 [29%]), or any posterior uveal invasion with any optic nerve involvement (24 [46%]). There was additional invasion into the sclera (3 [6%]) and extrascleral structures, including the orbit (1 [2%]). A single histopathologic high-risk factor was present in 32 eyes (62%), whereas 20 eyes (38%) manifested 2 or more highrisk characteristics. Based on previously published series, untreated high-risk retinoblastoma carries at least a 24% risk for metastatic disease. In the present series, using vincristine, etoposide, and carboplatin in all cases, there was no metastasis during a mean follow-up of 66 months (median [range], 55 months).Conclusions: Retinoblastoma with invasion into the postlaminar optic nerve and/or posterior uvea is at high risk for metastasis and death. In this study, postenucleation chemotherapy using vincristine, etoposide, and carboplatin was effective in preventing metastasis in every case (100%).

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“…Khelfaoui et al, com base no exame histopatológico e evolução clínica de 172 pacientes, propuseram a divisão em 5 grupos para invasão de túnicas oculares e 4 para a invasão de nervo óptico (Quadro I). Segundo esses autores, a invasão de nervo óptico além da lâmina crivosa e/ou invasão maciça de coróide estariam associados a uma maior freqüência de desenvolvimento de doença disseminada após enucleação (8) . O tratamento depende do estadiamento da lesão, sendo que a enucleação permanece como a forma mais comum de tratamento (4) .…”

Section: Achados Histopatológicos Em Retinoblastomaunclassified