- Volume 15 Issue 18
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Presentation of Retinoblastoma Patients in Malaysia
- Subramaniam, Shridevi (National Clinical Research Centre, Dermatology Block, Hospital Kuala Lumpur) ;
- Rahmat, Jamalia (Department of Ophthalmology, Hospital Kuala Lumpur) ;
- Rahman, Norazah Abdul (Department of Ophthalmology, Hospital Kuala Lumpur) ;
- Ramasamy, Sunder (Department of Ophthalmology, Hospital Kuala Lumpur) ;
- Bhoo-Pathy, Nirmala (National Clinical Research Centre, Dermatology Block, Hospital Kuala Lumpur) ;
- Pin, Goh Pik (National Clinical Research Centre, Dermatology Block, Hospital Kuala Lumpur) ;
- Alagaratnam, Joseph (Department of Ophthalmology, Hospital Kuala Lumpur)
- Published : 2014.10.11
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.
- Abiose A, Adido J, Agarwal SC (1985). Childhood malignancies of the eye and orbit in Northern Nigeria. Cancer, 55, 2889-93. https://doi.org/10.1002/1097-0142(19850615)55:12<2889::AID-CNCR2820551232>3.0.CO;2-5
- Arif M, Iqbal Z, Zia-ul-Islam (2009). Retinoblastoma in NWFP, Pakistan. J Ayub Med Coll Abbottabad, 21, 60-2.
- Abramson DH, Beaverson K, Sangani P, et al (2003). Screening for retinoblastoma: presenting signs as prognosticators of patients and ocular survival. Pediatr, 112, 1248-55. https://doi.org/10.1542/peds.112.6.1248
- Abramson DH, Ellsworth RM, Kitchin FD, Tung G (1984). Second non-ocular tumours in retinoblastoma surviviors: Are they radiation induced? Opthalmology, 91, 1351-5. https://doi.org/10.1016/S0161-6420(84)34127-6
- Ajaiyeoba IA, Pindiga HU, Akang EE (1992). Tumours of the eye and orbit in ibadan. East Afr Med J, 69, 487-9.
- Aung L, Chan YH, Yeoh EJ, Tan PL, Quah TC (2009). Retinoblastoma: a recent experience at the national university hospital, Singapore. Ann Acad Med Singapore, 38, 693-8.
- Bakshi S, Gupta S, Gogia V, Ravindranath Y (2010). Compliance of retinoblastoma. Indian J Pediatr, 77, 535-40. https://doi.org/10.1007/s12098-010-0047-8
- Balasubramanya R, Pushker N, Bajaj MS, et al (2004). Atypical presentations of retinoblastoma. J Pediatr Ophthalmol Strabismus, 41, 18-24.
- Bonanomi MT, Almeida MT, Cristofani LM, Odone Filho V (2009). Retinoblastoma: A three-year-study at a Brazilian medical school hospital. Clinic, 64, 427-34.
- Bukhari S, Rehman A, Bhutto IA, Qidwai U (2011). Presentation pattern of retinoblastoma. Pak J Opthalmol, 27, 142-5.
- Canzano JC, Handa JT (1999). Utility of pupillary dilation for detecting leukocoria in patients with retinoblastoma. Pediatric, 104, 44. https://doi.org/10.1542/peds.104.4.e44
- Chan HS, Gallie BL, Munier FL, Beck Popovic M (2005). Chemotherapy for retinoblastoma. Ophthalmol Clin North Am, 18, 55-63. https://doi.org/10.1016/j.ohc.2004.11.002
- Ellsworth RM (1969). The practical management of retinoblastoma. Trans Am Ophthalmol Soc, 67, 462-34.
- Chantada G, Fandino A, Manzitti J, Urrutia L, Schvartzman E (1999). Late diagnosis in a developing country. Arch Dis Child, 80, 171-4. https://doi.org/10.1136/adc.80.2.171
- Carter J (2009). Recognizing the signs of retinoblastoma. Practice Nursing, 20, 394-7. https://doi.org/10.12968/pnur.2009.20.8.43658
- Dudgeon J (1995). Retinoblastoma-trends in conservative management. Br J Opthalmol, 79, 104. https://doi.org/10.1136/bjo.79.2.104
- Essuman V, Ntim-Amponsah CT, Akafo SK, Renner L, Edusei L (2010). Presentation of retinoblastoma at a paediatric eye clinic in Ghana. Ghana Med J, 44, 10-5.
- Hathirat P, Kunavisarut S, Chuansumrit A, et al (1993). Chemotherapy in patients with retinoblastoma. J Med Assoc Thai, 76, 85-91.
- Jamalia R, Sunder R, Alagaratnam J, PP Goh (2010). Retinoblastoma registry report-Hospital Kuala Lumpur Experience. Med J Malaysia, 65, 128-30.
- Kaimbo WK, Mvitu MM, Missotten L (2002).Presenting signs of retinoblastoma in Congolese patients. Bull Soc Belge Ophthalmol, 283, 37-41.
- Klauss V (1990). Retinoblastoma in Developing Countries. Community Eye Health, 5, 1-2.
- Klauss V, Chana HS (1983). Ocular tumours in Africa. Soc Sci Med, 17, 1743-50. https://doi.org/10.1016/0277-9536(83)90386-6
- Nafianti S (2006). Retinoblastoma in children in Haji Adam Malik hospital Medan. Majalah Kedokteran Nusantara, 39, 147-50.
- Owoeye JF, Afolayan EA, Ademola-Popoola DS (2006). Retinoblastoma-a clinic-pathological study in Ilorin, Nigeria. Afr J Health Sci, 13, 117-23.
- O'Brien JM (2001). Retinoblastoma: clinical presentation and the role of neuroimaging. AJNR Am J Neuroradiol, 22,427-9.
- PP Goh, Elias H, NorFariza N, Mariam I (2008). A Web Based Surveillance System. Med J Malaysia, 63, 20-23.
- Parkin DM, Kramarova E, Draper GJ, et al., editors (1998). International incidence of childhood cancer: volume 2. IARC Scientific Publications No 144. Lyon: IARC.
- Parkin DM, Stiller CA, Draper GJ, Bieber CA (1988). The international incidence of childhood cancer. Int J Cancer, 42, 511-20. https://doi.org/10.1002/ijc.2910420408
- Patikulsila P, Patikulsila D (2001). Retinoblastoma at Maharaj Nakorn Chang Mai Hospital; a 7 year study. Changmai Med Bull, 40, 167-72.
- Reddy SC, Anusya S (2010). Clinical presentation of retinoblastoma in Malaysia: a review of 64 patients. Int J Ophthalmol, 3, 64-8
- Sahul S, Banavali SD, Pai SK, et al (1998). Retinoblastoma: Problems and Perspectives from India. Pediat Haema Oncology, 15, 501-8. https://doi.org/10.3109/08880019809018311
- Sanders BM, Draper GJ, Kingston JE (1988). Retinoblastoma in Great Britain 1969-80: incidence, treatment, and survival. Br J Ophthalmol, 72, 576-83. https://doi.org/10.1136/bjo.72.8.576
- Sang DN, Albert DM (1982). Retinoblastoma: clinical and histopathologic features. Human Pathol, 13, 133-47. https://doi.org/10.1016/S0046-8177(82)80117-2
- Sitorus RS, Moll AC, Suhardjono S, et al (2009). The effects of therapy refusal against medical advice in retinoblastoma patients in a setting where treatment delays are common. Ophthalmic Genet, 30, 31-6. https://doi.org/10.1080/13816810802464320
- Wallach M, Balmer A, Munier F, et al (2006). Shorter time to diagnosis and improved stage at presentation in Swiss patients with retinoblastoma treated from 1963 to 2004. Pediatrics, 118, 1493-8. https://doi.org/10.1542/peds.2006-0854
- Wiangnon S, Kamsa-ard S, Jetsrisuparb A, et al (2003). Childhood cancer in Thailand 1995-1997. Asian Pac J Cancer Prev, 4, 337-43.
- Zelter M, Gonzalez G, Schwartz L, et al (1988). Treatment of retinoblastoma. Cancer, 61, 153-60. https://doi.org/10.1002/1097-0142(19880101)61:1<153::AID-CNCR2820610126>3.0.CO;2-#
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- Retinoblastoma: A Global Perspective vol.5, pp.2, 2017, https://doi.org/10.1007/s40135-017-0138-z
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