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CC BY-NC-ND 4.0 · South Asian J Cancer 2023; 12(02): 199-205
DOI: 10.1055/s-0042-1757581
Original Article
Pediatrics

Epidemiological and Clinical Presentation of Retinoblastoma among Nepalese Children in 2019

Ben Limbu 
1   Department of Oculoplastic and Reconstructive Surgery, Tilganga Institute of Ophthalmology, National Academy of Medical Sciences, Kathmandu, Nepal
,
Prerna Arjyal Kafle
2   Department of Oculoplastic and Reconstructive Surgery, Biratnagar Eye Hospital, Biratnagar, Nepal
,
Diwa Hamal Lamichanne
2   Department of Oculoplastic and Reconstructive Surgery, Biratnagar Eye Hospital, Biratnagar, Nepal
,
Suresh Raj Pant
3   Department of Oculoplastic and Reconstructive Surgery, Geta Eye Hospital, Attariya, Nepal
,
Samyek Shakya
4   Department of Vitreoretinal Surgery, Geta Eye Hospital, Attariya, Nepal
,
Suresh B.K. Rasaily 
4   Department of Vitreoretinal Surgery, Geta Eye Hospital, Attariya, Nepal
› Author Affiliations Funding This study was supported financially and logistically by Nepalese Society of Oculoplastic Surgeons (NESOS) and KAKS foundation.
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Abstract

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Suresh B.K. Rasaily

Objectives Retinoblastoma (RB) is rare but potentially fatal if left untreated. This study aimed to evaluate the epidemiological profile, clinical manifestation, classification, and affordability of RB treatment among Nepalese children in the year 2019.

Materials and Method A multicentric, multiethnic hospital-based cross-sectional study after ethical approval from the National Health Research Council was conducted from January 2019 to December 2019 by incorporating ophthalmologists all over the nation. Twenty-seven RB centers were selected. All the RB presented in the RB centers either newly diagnosed or ongoing treatment consented to the study were included and failed to consent for the study, RB survivors were excluded from the study. Data based on demographic profile, clinical manifestation, ethnical and geographical distribution, and treatment received were collected in the customized Google Form. Each case was classified at the time of diagnosis as per the International Classification of Retinoblastoma groups and different treatment modalities offered as per grouping and staging. The affordability of treatment was calculated using a catastrophic approach.

Statistical Analysis Data were entered into Microsoft Excel 2010 and analyzed using Statistical Package for Social Sciences version 20.

Result A total of 34 RB cases, 21 (61.76%) in ongoing treatment group and 13 (38.2%) in newly diagnosed group presented in the RB centers. Out of total, 32 (64.7%) had unilateral and 12 (35.3%) cases had bilateral involvement. The majority of patients was from Province 1 (35.3%) and belonged to the upper caste (38.2%). Leukocoria was the most common presentation (73.9%) followed by proptosis, red eye, and phthisis bulbi. More than 75% patients presented at advanced group D (54%) and E (21%) and stage 0 (90%). More than 90% of patients received systemic chemotherapy, and 42.6% received transpupillary thermotherapy. The average cost of RB treatment was estimated to be 521% of the nonfood expense of the family which is unaffordable to almost all cases (100%).

Conclusion Leukocoria is the most common mode of clinical presentation in both unilateral and bilateral RBs. Early diagnosis and appropriate treatment are key to success for saving life, sight, and eye. However, community awareness programs against RB, active referral networks, and the establishment of chemotherapy centers with trained human resources are needed to reduce loss of life, sight, and eye.

Keywords

affordability - chemotherapy - clinical presentation - epidemiology - ethnicity - retinoblastoma

Ethical Approval

Ethical approval from National Health Research Council (NHRC) Ethical Review Board (Ref. no 1768).


The authors declared that the study was conducted in accordance with the tenets of Declaration of Helsinki.


Authors' Contributions

B.L. played a pivotal role in conceptualizing the study, designing it, and contributing to the manuscript's written content, along with performing statistical analysis. P.A. also made significant contributions to manuscript writing, statistical analysis, and interpretation. D.H. actively participated in manuscript writing, manuscript review, statistical analysis, and interpretation of results. S.R.P. was involved in writing, reviewing the manuscript, and performing statistical analysis. S.S. contributed to both writing and manuscript review, as well as analyzing the results. Lastly, S.B.K.R. was instrumental in manuscript writing and design, critically analyzing the results, and providing valuable input during manuscript review. Our collective efforts have culminated in this comprehensive piece of work.




Publication History

Article published online:
02 November 2022

© 2022. MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Reddy SC, Anusya S. Clinical presentation of retinoblastoma in Malaysia: a review of 64 patients. Int J Ophthalmol 2010; 3 (01) 64-68
    PubMedGoogle Scholar
  • 2 Naseripour M. “Retinoblastoma survival disparity”: the expanding horizon in developing countries. Saudi J Ophthalmol 2012; 26 (02) 157-161
    CrossrefPubMedGoogle Scholar
  • 3 Fabian ID, Abdallah E, Abdullahi SU. et al; Global Retinoblastoma Study Group. Global retinoblastoma presentation and analysis by national income level. JAMA Oncol 2020; 6 (05) 685-695
    CrossrefPubMedGoogle Scholar
  • 4 Chantada GL, Qaddoumi I, Canturk S. et al. Strategies to manage retinoblastoma in developing countries. Pediatr Blood Cancer 2011; 56 (03) 341-348
    CrossrefPubMedGoogle Scholar
  • 5 Dimaras H, Kimani K, Dimba EAO. et al. Retinoblastoma. Lancet 2012; 379 (9824): 1436-1446
    CrossrefPubMedGoogle Scholar
  • 6 Sah KP, Saiju R, Roy P, Kafle S. Retinoblastoma: ten years experience at Kanti Children's Hospital. JNMA J Nepal Med Assoc 2013; 52 (192) 576-579
    PubMedGoogle Scholar
  • 7 Ben L, Aemero M, Gushchin AG, Moore GH, Rohit S. Clinico-pathological patterns of patients who underwent orbital exenteration in a tertiary eye hospital of Nepal. Ethiop J Health Sci 2016; 26 (06) 543-548
    CrossrefPubMedGoogle Scholar
  • 8 Saiju R, Moore G, Shrestha U, Shrestha MK, Ruit S. Retinoblastoma: geographic distribution and presentation at a tertiary eye care centre in Kathmandu, Nepal. Nepal J Ophthalmol 2013; 5 (02) 169-176
    CrossrefPubMedGoogle Scholar
  • 9 Gurung HBLB. Lama AJBH, Saiju R. Retinoblastoma: changing trend in a tertiary eye center. Indian J Clin Exp Ophthalmol 2019; 5 (April-June): 252-256
    CrossrefGoogle Scholar
  • 10 Health Management Information System Ethnic Code. Accessed September 19, 2022, at: https://plos.figshare.com/articles/dataset/_Ethnic_codes_as_defined_by_the_Health_Management_Information_System_/767015/1
  • 11 Shields CL, Shields JA. Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophthalmol 2006; 17 (03) 228-234
    CrossrefPubMedGoogle Scholar
  • 12 Goddard AG, Kingston JE, Hungerford JL. Delay in diagnosis of retinoblastoma: risk factors and treatment outcome. Br J Ophthalmol 1999; 83 (12) 1320-1323
    CrossrefPubMedGoogle Scholar
  • 13 Seth R, Singh A, Guru V, Chawla B, Pathy S, Sapra S. Long-term follow-up of retinoblastoma survivors: experience from India. South Asian J Cancer 2017; 6 (04) 176-179
    Article in Thieme ConnectPubMedGoogle Scholar
  • 14 Akhiwu WO, Igbe AP. Epidemiology of retinoblastoma. In: Retinoblastoma: An Update on Clinical, Genetic Counseling, Epidemiology and Molecular Tumor Biology. IntechOpen 2012
    PubMedGoogle Scholar
  • 15 Rangamani S, SathishKumar K, Manoharan N. et al. Paediatric retinoblastoma in India: evidence from the National Cancer Registry Programme. Asian Pac J Cancer Prev 2015; 16 (10) 4193-4198
    CrossrefPubMedGoogle Scholar
  • 16 Zhao J, Li S, Shi J, Wang N. Clinical presentation and group classification of newly diagnosed intraocular retinoblastoma in China. Br J Ophthalmol 2011; 95 (10) 1372-1375
    CrossrefPubMedGoogle Scholar
  • 17 Naseripour M, Nazari H, Bakhtiari P, Modarres-zadeh M, Vosough P, Ausari M. Retinoblastoma in Iran: outcomes in terms of patients' survival and globe survival. Br J Ophthalmol 2009; 93 (01) 28-32
    CrossrefPubMedGoogle Scholar
  • 18 Chung SE, Sa HS, Koo HH, Yoo KH, Sung KW, Ham DI. Clinical manifestations and treatment of retinoblastoma in Korea. Br J Ophthalmol 2008; 92 (09) 1180-1184
    CrossrefPubMedGoogle Scholar
  • 19 MacCarthy A, Birch JM, Draper GJ. et al. Retinoblastoma in Great Britain 1963-2002. Br J Ophthalmol 2009; 93 (01) 33-37
    CrossrefPubMedGoogle Scholar
  • 20 Chen YH, Lin HY, Hsu WM, Lee SM, Cheng CY. Retinoblastoma in Taiwan: incidence and survival characteristics from 1979 to 2003. Eye (Lond) 2010; 24 (02) 318-322 (Lond Engl)
    CrossrefPubMedGoogle Scholar
  • 21 Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda VAR. Retinoblastoma in India: clinical presentation and outcome in 1,457 patients (2,074 eyes). Retina 2019; 39 (02) 379-391
    CrossrefPubMedGoogle Scholar
  • 22 Kazadi Lukusa A, Aloni MN, Kadima-Tshimanga B. et al. Retinoblastoma in the democratic republic of Congo: 20-year review from a tertiary hospital in Kinshasa. J Cancer Epidemiol 2012; 2012: 920468
    CrossrefPubMedGoogle Scholar
  • 23 Pedram M, Alghasi A, Salari F. et al. Retinoblastoma in southwest Iran. Iran J Blood Cancer 2015; 7 (03) 143-148
    Google Scholar
  • 24 Azar D, Donaldson C, Kalapesi F, Cumming R. Retinoblastoma in New South Wales 1975 to 2001. J Pediatr Hematol Oncol 2006; 28 (10) 642-646
    CrossrefPubMedGoogle Scholar
  • 25 Badhu B, Sah SP, Thakur SK. et al. Clinical presentation of retinoblastoma in Eastern Nepal. Clin Exp Ophthalmol 2005; 33 (04) 386-389
    CrossrefPubMedGoogle Scholar
  • 26 Chuluunbat T, Jamiyanjav B, Munkhuu B. et al. Retinoblastoma in Mongolia: clinical characteristics and survival from 1987 to 2014. Taiwan J Ophthalmol 2016; 6 (02) 79-84
    CrossrefPubMedGoogle Scholar
  • 27 Sahu S, Banavali SD, Pai SK. et al. Retinoblastoma: problems and perspectives from India. Pediatr Hematol Oncol 1998; 15 (06) 501-508
    CrossrefPubMedGoogle Scholar
  • 28 Harini R, Ata-ur-Rasheed M, Shanmugam MP, Amali J, Das D, Kumaramanickavel G. Genetic profile of 81 retinoblastoma patients from a referral hospital in southern India. Indian J Ophthalmol 2001; 49 (01) 37-42
    PubMedGoogle Scholar
  • 29 Butros LJ, Abramson DH, Dunkel IJ. Delayed diagnosis of retinoblastoma: analysis of degree, cause, and potential consequences. Pediatrics 2002; 109 (03) E45
    CrossrefPubMedGoogle Scholar
  • 30 Miranda GA, Simanjuntak GWS. Clinical findings and demography of retinoblastoma in a tertiary hospital in a remote area in a developing country. Asia Pacific J Cancer Care 2018; 3 (02) 37
    CrossrefGoogle Scholar
  • 31 Lim FP, Soh SY, Iyer JV, Tan AM, Swati H, Quah BL. Clinical profile, management, and outcome of retinoblastoma in Singapore. J Pediatr Ophthalmol Strabismus 2013; 50 (02) 106-112
    CrossrefPubMedGoogle Scholar
  • 32 El Zomor H, Nour R, Alieldin A. et al. Clinical presentation of intraocular retinoblastoma; 5-year hospital-based registry in Egypt. J Egypt Natl Canc Inst 2015; 27 (04) 195-203
    CrossrefPubMedGoogle Scholar
  • 33 Kaliki S, Srinivasan V, Gupta A, Mishra DK, Naik MN. Clinical features predictive of high-risk retinoblastoma in 403 Asian Indian patients: a case-control study. Ophthalmology 2015; 122 (06) 1165-1172
    CrossrefPubMedGoogle Scholar
  • 34 Saiju R, Thakur J, Karmacharya PC, Shah DN. Retinoblastoma in Nepal: a clinical profile of 30 cases. Nepal Med Coll J 2006; 8 (03) 171-175
    PubMedGoogle Scholar
  • 35 Wilimas JA, Wilson MW, Haik BG. et al. Development of retinoblastoma programs in Central America. Pediatr Blood Cancer 2009; 53 (01) 42-46
    CrossrefPubMedGoogle Scholar