Retinoblastoma in Taiwan: Survival Rate and Prognostic Factors

Chang, Ching-Yun; Chiou, Tzeon Jye; Hwang, Betau; Li, Bai; Hsu, Wen Ming; Hsieh, Yuh Lin

doi:10.1007/s10384-005-0320-y

Cited by 62 publications

(68 citation statements)

References 11 publications

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“…In the present study, the mean age at diagnosis of Rb was 24.5 months, which was similar to that reported in other developing countries and regions (24-28.5 months) (9)(10)(11)20) and in between the mean age in developed countries (19.7 months) (17) and less developed countries (36.5 months) (23) . Navo et al (20) found that death could be maximally avoided in younger Rb patients with less lag time before treatment if the patients were diagnosed early.…”

Section: Discussionsupporting

confidence: 90%

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Luo¹,

Ye²,

Ding³

et al. 2015

Turk J Med Sci

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IntroductionRetinoblastoma (Rb) is the most common intraocular malignant tumor in children, accounting for approximately 3%-4% of all malignant tumors in children aged 0-14 years (1,2). Currently, in developed countries with advanced tools for medical diagnosis, Rb can be detected early, thus leading to an improved survival rate of more than 95% for this high-mortality disease. However, the survival rate from this disease is still as low as approximately 50% worldwide (3). This may be due to the poor diagnostic and therapeutic capacity, as well as generally poor health conditions, experienced in low-income countries. This leads to late diagnosis of the disease and thus a high incidence of extraocular complications and a high risk of distant metastasis (4). Rb can be cured if the disease is diagnosed early, if disease severity is appropriately assessed, and if proper treatments are applied (5). Therefore, this study aimed to identify clinical characteristics of Rb patients, to find the potential relationship between clinical manifestations and survival rates from Rb, and to provide a clinical basis for prediction of disease outcomes and treatment options. Materials and methods Clinical dataThis retrospective study with complete follow-up data included 314 patients who were diagnosed as having Rb at the Zhongshan Ophthalmic Center at Sun Yat-sen University from January 2003 to February 2011. Clinical data, including laterality of eyes, sex, age at diagnosis, presenting signs, lag time before treatment, survival conditions, and survival time, were recorded. Patients were followed until death or the cutoff date (February 2013), whichever occurred first. The mean follow-up time was 41.4 ± 27.9 months (median, 33 months; range, 1-110 months).Patients' clinical presentations at diagnosis were categorized into 6 groups based on the description given by the patients or their families: 1) leukocoria (white reflection from the pupil); 2) strabismus; 3) inflammation Background/aim: The aim of this study was to investigate the potential correlation between clinical characteristics and prognosis of patients with retinoblastoma (Rb). Materials and methods:This retrospective study included 314 Rb patients. Clinical data including laterality of eyes, sex, age, presenting signs, lag time, and survival were recorded and analyzed.Results: Leukocoria is the most common clinical presentation of Rb. Patients with isolated leukocoria had shorter lag time and exhibited a high survival rate (85%, 5 years). Patients with strabismus and blurred vision, and who were older and had longer lag time, exhibited an excellent survival rate (100% and 92.3%, respectively, 5 years). Patients with exophthalmos had the longest lag time and the lowest survival rate (17.8%, 5 years). The 5-year survival rate of patients with a lag time of ≤6 months was 84.7%, which was significantly higher than that of patients with a lag time of >6 months (64.7%). Conclusion:Leukocoria, strabismus, and blurred vision are mild clinical manifestations of Rb that are associ...

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Section: Discussionsupporting

confidence: 90%

Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

Luo¹,

Ye²,

Ding³

et al. 2015

Turk J Med Sci

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“…3,4,5,6 However, in developing countries, the majority of patients with retinoblastoma present with advanced disease with the resultant 5 year survival of less than 50%. 7,8,9 The factors for this poor prognosis may include late presentation with a high mean age at diagnosis, 10,11 interval between onset and treatment of more than 5 months, a lag time before treatment exceeding 2.5 months, extraocular disease, 12 higher occurrence of poorly differentiated tumours, 13 presence of other histopathological high risk features (HHRF) for metastasis, and other factors such as lack of knowledge about the disease and its outcome, high costs of hospital care (diagnosis and treatment), and lack of resources for care. Improvement in survival in developing countries will require measures that enhance early reporting through health education, 14 early and cheaper means of screening those at risk and early diagnosis of affected patients, as well as early, affordable and effective forms of treatment including means of funding such as national health insurance or government-funded schemes.…”

Section: Introductionmentioning

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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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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“…In our study, median age of presentation was 24 months which is comparable with previous other studies from North India (29 months), Iran (28.5 months), Taiwan (26.3 months) and Malaysia (24.2 months). 15,20,21 A relatively higher median age at diagnosis has been reported from African countries like Ghana (36 months) and Kenya (37.5 months). 14,22 Tumours were relatively diagnosed at an earlier age in Australia and USA.…”

Section: Discussionmentioning

confidence: 97%

“…12 Stage of the disease had a significant impact on the survival outcomes, which is consistent with previous studies that reported an association between severity of disease and the survival outcome. 21,33,34 The most feared complication of chemotherapy is the risk of developing other tumors in the medium and long term, particularly leukemia by use of Etoposide. 35 Radiation appears to have a higher incidence of non-ocular tumours.…”

Section: Discussionmentioning

confidence: 99%