Retinoblastoma in Turkey: survival and clinical characteristics 1981–2004

Özkan, Alp; Pazarlı, Halit; Çelkan, Tiraje; Karaman, Serap; Apak, Hilmi; Kaner, Gültekin; Uzel, Ömer; Yıldız, İnci

doi:10.1111/j.1442-200x.2006.02223.x

Cited by 37 publications

(34 citation statements)

References 18 publications

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“…This conforms with studies from both developed and developing countries although this proportion seems higher in our small series. 9,10,16 The mean age of diagnosis was high at 36.3 months. This finding confirms observations from other developing countries in Africa and the Asia.…”

Section: Discussionmentioning

confidence: 98%

“…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. 15,16,17 At the Paediatric Department of Ghana's premier teaching hospital, Korle Bu Teaching Hospital, Accra, retinoblastoma was found to be the second commonest childhood tumour after Burkitt's lymphoma. 18 Retinoblastoma was the commonest malignant intraocular childhood cancer seen at the Eye Clinic of the same hospital, and as elsewhere, and formed about 50% of ocular tumours seen at the clinic.…”

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: 98%

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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“…Pathologic evaluation of enucleated eyes is seldom done, and relatively intensive chemotherapy is not usually available [19][20][21]. Survival in many middle-income countries (MICs) is over 70%, and some upper MICs that have established multidisciplinary teams and adequate treatment facilities have survival rates near 90% (Table I) [22][23][24][25].…”

Section: Outcome Of Retinoblastoma In Developing Countriesmentioning

confidence: 99%

“…In contrast, in most MICs, metastatic disease is present at diagnosis in less than 10% of the cases [7,22,23]. Treatment with neoadjuvant chemotherapy, followed by secondary enucleation and adjuvant therapy is the current approach in most centers [13,40,41].…”

Section: Treating Patients With Overt Extraocular Diseasementioning

confidence: 99%

“…Such cases are more frequent in developing countries, occurring in more than 50% of children in some MICs compared to developed countries where they usually occur in less than 20% of the enucleated eyes. Studies in many upper MICs have yielded encouraging results, with survival rates greater than 80% for patients with these conditions [23,45,46]. However, as opposed to other childhood malignancies, there are no randomized studies to support the treatment decisions in these cases.…”

Section: Treating Patients With High-risk Pathology Featuresmentioning

confidence: 99%

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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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Lack of Tir Ubiquitylation Contributes to Enteropathogenic E. coli Remaining Extracellular During Nonphagocytic Cell Infections

Lin

Guttman

2012

The Anatomical Record

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Enteropathogenic Escherichia coli (EPEC) is an extracellular pathogen that alters many host subcellular components during its infectious processes. We have previously shown that EPEC hijacks a large assortment of host cell endocytic components and uses these proteins to form protruding structures called ''pedestals'' rather than triggering internalization of the bacteria. Other invasive pathogens that also recruit similar endocytic components have been shown to enter their host cells on the ubiquitylation of their host cell receptors. Therefore, we hypothesize that EPEC remains extracellular by maintaining its receptor, translocated intimin receptor (Tir), in an unubiquitylated state. Using immunoprecipitation-Western blots, we demonstrate no association of ubiquitin with Tir. To further elucidate the effect Tir ubiquitylation would have on EPEC during their infections, we engineered Tir-ubiquitin fusion constructs, expressed them in host epithelial cells, and infected them with Dtir EPEC. We found these cells induced a significant increase in EPEC invasion as compared with cells that expressed the Tir construct that lacked ubiquitin conjugation. Our results indicate that the lack of EPEC receptor ubiquitylation is a contributing factor that these microbes use to prevent their internalization into epithelial cells.

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Retinoblastoma in Turkey: survival and clinical characteristics 1981–2004

Abstract: The study's survival rate was similar to developed countries. The success in higher survival rates is based on the authors multidisciplinary team approach done by the same group and the support of the authors' clinic and government in sponsoring the medical insurance of all patients.

Cited by 37 publications

References 18 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

Lack of Tir Ubiquitylation Contributes to Enteropathogenic E. coli Remaining Extracellular During Nonphagocytic Cell Infections

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