MyMedR

Displaying all 7 publications

Abstract:

Sort:

Fulltext Global Retinoblastoma Presentation and Analysis by National Income Level

Global Retinoblastoma Study Group, Fabian ID, Abdallah E, Abdullahi SU, Abdulqader RA, Adamou Boubacar S, et al.

JAMA Oncol, 2020 May 01;6(5):685-695.
PMID: 32105305 DOI: 10.1001/jamaoncol.2019.6716

IMPORTANCE: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.
OBJECTIVES: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis.
DESIGN, SETTING, AND PARTICIPANTS: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017.
MAIN OUTCOMES AND MEASURES: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis.
RESULTS: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]).
CONCLUSIONS AND RELEVANCE: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

Matched MeSH terms: Retinoblastoma/epidemiology*
Fulltext Retinoblastoma Registry report--Hospital Kuala Lumpur experience

Jamalia R, Sunder R, Alagaratnam J, Goh PP

Med J Malaysia, 2010 Jun;65 Suppl A:128-30.
PMID: 21488473

Retinoblastoma is a childhood ocular cancer. The aim of this paper is to describe the clinical and epidemiological characteristics of patients with retinoblastoma in a major paediatric ophthalmology center in the country. Retrospective information was collected through the retinoblastoma registry. Late presentation with advanced staging is a major problem.

Matched MeSH terms: Retinoblastoma/epidemiology
Extraocular retinoblastoma

Menon BS, Reddy SC, Maziah WM, Ham A, Rosline H

Med. Pediatr. Oncol., 2000 Jul;35(1):75-6.
PMID: 10881012
Matched MeSH terms: Retinoblastoma/epidemiology*
Advanced retinoblastoma in Malaysian children

Sinniah D, Narasimha G, Prathap K

Acta Ophthalmol, 1980 Oct;58(5):819-24.
PMID: 7211270

Twenty children with retinoblastoma are reviewed who were treated at the University Hospital, Kuala Lumpur over a 10-year-period. They constitute 6.6% of childhood malignancies and without exception all presented with advanced disease. Hereditary cases were notably absent in the the series probably because past cases have almost invariably succumbed without an opportunity to transmit the gene. With enucleation and radiotherapy six of the patients have survived from 2 to 12 years. The addition of vincristine and cyclophosphamide has not been associated with improved survival.

Matched MeSH terms: Retinoblastoma/epidemiology*
Fulltext Presentation of retinoblastoma patients in Malaysia

Subramaniam S, Rahmat J, Rahman NA, Ramasamy S, Bhoo-Pathy N, Pin GP, et al.

Asian Pac J Cancer Prev, 2014;15(18):7863-7.
PMID: 25292078

BACKGROUND: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia.

MATERIALS AND METHODS: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications.

RESULTS: The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012. There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majority of patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%). Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It was found that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis (12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%). More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients.

CONCLUSIONS: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

Matched MeSH terms: Retinoblastoma/epidemiology*
RB pocket domain B mutation frequency in Malaysia

Ishak SR, Hanafi H, Alagaratnam JV, Zilfalil BA, Tajudin LS

Ophthalmic Genet., 2010 Sep;31(3):159-61.
PMID: 20565234 DOI: 10.3109/13816810.2010.492816
Matched MeSH terms: Retinoblastoma/epidemiology
Late presentation of retinoblastoma in Malaysia

Menon BS, Alagaratnam J, Juraida E, Mohamed M, Ibrahim H, Naing NN

Pediatr Blood Cancer, 2009 Feb;52(2):215-7.
PMID: 18855905 DOI: 10.1002/pbc.21791

The aims of this study were to review the presenting features, treatment and outcome for Malaysian children with retinoblastoma currently.

Matched MeSH terms: Retinoblastoma/epidemiology