Metastatic RetinoblastomaClinical Features, Treatment, and Prognosis

Günduz, Kaan; Müftüoğlu, Orkun; Günalp, İlhan; Ünal, Emel; Taçyıldız, Nurdan

doi:10.1016/j.ophtha.2006.03.039

Cited by 99 publications

(53 citation statements)

References 35 publications

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“…9,10,20 A higher mean age at diagnosis has been found to be associated with advanced or metastatic retinoblastoma. 21 This is in sharp contrast with the findings from developed countries, where the mean age of diagnosis is less than 24 months. 5 The common clinical presentations of leukocoria, proptosis squint and red eye from this study are also comparable to those in other developing countries.…”

Section: Discussionmentioning

confidence: 62%

“…10,11,[22][23][24] These features together with other signs such as, secondary glaucoma, fungating mass, hyphaema and flattened anterior chamber are known signs of advanced disease or high risk for metastasis. 2,23 A study of 29 patients in Congo revealed the common presenting signs to be leukocoria, 49%, followed by proptosis (28%). Other signs were strabismus, red eye, anterior scleral staphyloma, hyphema and buphthalmia.…”

Section: Discussionmentioning

confidence: 99%

“…As survival in patients with retinoblastoma is inversely related to the stage of the disease at presentation, [20][21][22][23][24] we carried out this study to determine the stages of retinoblastoma in our patients at presentation and their relation to outcome. This will serve as baseline data for further studies and help plan interventions for improvement in survival.…”

Section: Introductionmentioning

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: 62%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

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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“…Results of using conventional chemotherapy and irradiation for distant metastatic disease have been disappointing in developing countries [42,43]. Patients with metastatic disease outside the central nervous system (CNS) may be cured with high-dose chemotherapy and stem cell rescue [8,44]; however, these treatments usually are not available in LICs or most lower MICs.…”

Section: Treating Patients With Overt Extraocular Diseasementioning

confidence: 99%

Strategies to manage retinoblastoma in developing countries

Chantada

Qaddoumi

Canturk³

et al. 2010

Pediatric Blood & Cancer

131

109

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Survival of retinoblastoma is >90% in developed countries but there are significant differences with developing countries in stage at presentation, available treatment options, family compliance, and survival. In low‐income countries (LICs), children present with advanced disease, and the reasons are socioeconomic and cultural. In middle‐income countries (MICs), survival rates are better (>70%), but there is a high prevalence of microscopically disseminated extraocular disease. Programs for eye preservation have been developed, but toxicity‐related mortality is higher. Although effective treatment of microscopically extraocular disease improved the outcome, worldwide survival will be increased only by earlier diagnosis and better treatment adherence. Pediatr Blood Cancer 2011;56:341–348. © 2010 Wiley‐Liss, Inc.

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“…[3][4][5][6] Results from the most recent Latin-American series reporting patients treated with conventional chemo-and radiotherapy regimens were discouraging with survival figures from 0 to 20%. 3,7,8 Only occasional patients with distant metastatic disease survived and the few survivors reported with conventional chemotherapy included those who had metastasis limited to the lymph nodes, 9 which are now considered as regionally disseminated rather than a distant metastatic site.…”

Section: Introductionmentioning

confidence: 99%

Successful treatment of metastatic retinoblastoma with high-dose chemotherapy and autologous stem cell rescue in South America

Palma

Sasso

Dufort

et al. 2011

Bone Marrow Transplant

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High-dose chemotherapy (HDC) followed by autologous stem cell rescue (ASCR) is the only curative treatment for metastatic retinoblastoma, but its feasibility in developing countries is unknown. We report 11 consecutive children (six unilateral) treated in three South-American middleincome countries with HDC-ASCR. One patient had metastatic retinoblastoma at diagnosis and the remaining ones had a metastatic relapse. Metastatic sites included BM ¼ 6, bone ¼ 4, orbit ¼ 5 and central nervous system (CNS) ¼ 4. All patients received induction with conventional chemotherapy achieving CR at a median of 5.7 months from the diagnosis of metastasis. Conditioning regimens included carboplatin and etoposide with thiotepa in six or with CY in four or melphalan in one patient. All patients engrafted after G-CSF-mobilized peripheral blood ASCR and no toxic deaths occurred. Two children received post-ASCR CNS radiotherapy. Seven children have disease-free survival (median follow-up 39 months). CNS relapse, isolated (n ¼ 3) or with systemic relapse (n ¼ 1), occurring at a median of 7 months after ASCT was the most common event. In the same period, five children with metastatic retinoblastoma did not qualify for HDC-ASCR and died. We conclude that HDC-ASCR is a feasible and effective treatment for children with metastatic retinoblastoma in middle-income countries.

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Metastatic RetinoblastomaClinical Features, Treatment, and Prognosis

Cited by 99 publications

References 35 publications

Presentation of retinoblastoma at a paediatric eye clinic in Ghana

Presentation of retinoblastoma at a paediatric eye clinic in Ghana

Strategies to manage retinoblastoma in developing countries

Successful treatment of metastatic retinoblastoma with high-dose chemotherapy and autologous stem cell rescue in South America

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