Keywords: retinoblastoma, pediatric, clinical characteristics, management, survival

Background Studies on the clinical characteristics of retinoblastoma in Indonesia have been limited, even though retinoblastoma is the most common pediatric ocular malignancy worldwide and the second-most common pediatric malignancy in Indonesia. The estimated patient survival rate in lower-middle-income countries is 77%, and survival is highly dependent on early diagnosis and therapy.

Objective To identify the clinical characteristics of retinoblastoma in patients at the Cicendo National Eye Hospital, as an Indonesian tertiary eye hospital.

Methods This retrospective, observational study included pediatric patients <18 years of age diagnosed with retinoblastoma at the Cicendo National Eye Hospital, Bandung, West Java, between January 2018 and December 2022. We collected patients’ medical record data on age, gender, laterality, chief complaints, stage of disease, grouping, histopathologic features, therapy, and survival.

Results Of 247 retinoblastoma patients (328 eyes) enrolled, 53.85% presented at 1-2 years of age and 50.61% were males. Patients mostly presented with unilateral (67.21%) retinoblastoma, leukocoria (51.82%) as the chief complaint, and classified as International Retinoblastoma Staging System (IRSS) stage III (33.20%). Tumors were mostly intraocular (58.84%), particularly group E (39.02%). Most tumors showed undifferentiated (45.43%) and high-risk features (54.27%). Most eyes were treated with combination therapy (74.09%), particularly local and systemic therapy (59.45%).  Survival was noted at 18.62% of patients, but 62.75% of patients were lost to follow-up.

Conclusion Most patients present with advanced retinoblastoma. Most histopathological results shows undifferentiated and high-risk features of the tumor. Delays in diagnosis and therapy may occur due to a lack of awareness and knowledge regarding clinical manifestations of retinoblastoma, which leads to worse prognosis.

1. Sutaryo S, Kristian SD. A five-year review of children with neuroblastoma at Dr. Sardjito General Hospital, Yogyakarta, Indonesia. Paediatr Indones. 2019;59:157–63. DOI: https://doi.org/10.14238/PI59.3.2019.157-63
2. Pekacka A. The role of intraarterial chemotherapy in the management of retinoblastoma. J Ophthalmol. 2020;2020: 3638410. DOI: https://doi.org/10.1155/2020/3638410
3. Honavar SG, Rao R. Retinoblastoma. In: Nema HV, Nema N, editors. Ocular tumors. Singapore: Springer Singapore; 2021. p. 9–36.
4. Kaewkhaw R, Rojanaporn D. Retinoblastoma: etiology, modeling, and treatment. Cancers (Basel). 2020;12:2304. DOI: https://doi.org/10.3390/cancers12082304
5. Ancona-Lezama D, Dalvin LA, Shields CL. Modern treatment of retinoblastoma: a 2020 review. Indian J Ophthalmol. 2020;68:2356–65. DOI: https://doi.org/10.4103/ijo.IJO_721_20
6. Handayani K, Indraswari BW, Sitaresmi MN, Mulatsih S, Widjajanta PH, Kors WA, et al. Treatment outcome of children with retinoblastoma in a tertiary care referral hospital in Indonesia. Asian J Cancer Prev. 2021;22:1613–21. DOI: https://doi.org/10.31557/APJCP.2021.22.5.1613
7. Jain M, Rojanaporn D, Chawla B, Sundar G, Gopal L, Khetan V. Retinoblastoma in Asia. Eye. 2019;33:87–96. DOI: https://doi.org/10.1038/s41433-018-0244-7
8. U.S. Centers for Disease Control and Prevention. Positive Parenting Tips | Child Development | CDC [homepage on the Internet]. 2024 May [cited 2024 Aug 15]. Available from: https://www.cdc.gov/child-development/positive-parenting-tips/index.html
9. World Health Organization. Children: new threats to health [homepage on the Internet]. 2020 Nov [cited 2024 Aug 15]. Available from: https://www.who.int/news-room/fact-sheets/detail/children-new-threats-to-health
10. Ademola-Popoola DS, Opocher E, Reddy MA. Contemporary management of retinoblastoma in the context of a low-resource country. Niger Postgrad Med J. 2019;26:69–79. DOI: https://doi.org/10.4103/npmj.npmj_21_19
11. Kaliki S, Gupta S, Ramappa G, Mohamed A, Mishra DK. High-risk retinoblastoma based on age at primary enucleation: a study of 616 eyes. Eye. 2020;34:1441–8. DOI: https://doi.org/10.1038/s41433-019-0698-2
12. Zhou N, Yang L, Xu X, Liu Y, Wei W. Retinoblastoma in adults: clinical features, gene mutations and treatment outcomes: a study of six cases. Front Oncol. 2022;12:835965. DOI: https://doi.org/10.3389/fonc.2022.835965
13. Römer T, Temming P, Lohmann DR, Sturm D, von Deimling A, Sellhaus B, et al. Ectopic intracranial retinoblastoma in a 3.5-month-old infant without eye involvement and without evidence of heritability. Pediatr Blood Cancer. 2019;66:e27599. DOI: https://doi.org/10.1002/pbc.27599
14. Ueda T, Koga Y, Yoshikawa H, Tanabe M, Yamana K, Oba U, et al. Survival and ocular preservation in a long-term cohort of Japanese patients with retinoblastoma. BMC Pediatr 2020;20:37. DOI: https://doi.org/10.1186/s12887-020-1923-7
15. Abdelazeem B, Abbas KS, Shehata J, El-Shahat NA, Eltaras MM, Qaddoumi I, et al. Survival trends for patients with retinoblastoma between 2000 and 2018: What has changed? Cancer Med. 2022;12:6318–24. DOI: https://doi.org/10.1002/cam4.5406
16. Fabian ID, Khetan V, Stacey AW, Foster A, Ademola-Popoola DS, Berry JL, et al. Sex, gender, and retinoblastoma: analysis of 4351 patients from 153 countries. Eye (Lond). 2022;36:1571–7. DOI: https://doi.org/10.1038/s41433-021-01675-y
17. Global Retinoblastoma Study Group. Global retinoblastoma presentation and analysis by national income level. JAMA Oncol. 2020;6:685–95. DOI: https://doi.org/10.1001/jamaoncol.2019.6716
18. Chawla B, Hasan F, Azad R, Seth R, Upadhyay AD, Pathy S, et al. Clinical presentation and survival of retinoblastoma in Indian children. Br J Ophthalmol. 2016;100:172–8. DOI: https://doi.org/10.1136/bjophthalmol-2015-306672
19. Singh U, Katoch D, Kaur S, Dogra MR, Bansal D, Kapoor R. Retinoblastoma: a sixteen-year review of the presentation, treatment, and outcome from a tertiary care institute in Northern India. Ocul Oncol Pathol. 2017;4:23–32. DOI: https://doi.org/10.1159/000477408
20. Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda V. Retinoblastoma in India: clinical presentation and outcome in 1,457 patients (2,074 eyes). Retina. 2019;39:379–91. DOI: https://doi.org/10.1097/iae.0000000000001962
21. Da Silva FN, Kora LX, Elongo T, El kebir A, El maaloum L, Bennani-Guebessi N, et al. Anatomo-clinical aspects of retinoblastoma: a series of 144 cases. Cureus. 2022;14:e25422. DOI: https://doi.org/10.7759/cureus.25422
22. Domínguez-Varela IA, Aguilera-Partida JA, Dalvin LA, Garza-Garza LA, Thompson-García LM, Barbosa-Quintana Á, et al. Retinoblastoma in an older Hispanic child masquerading as pars planitis: A case report. Eur J Ophthalmol. 2022;32:NP71–4. DOI: https://doi.org/10.1177/1120672121994487
23. Chen PY, Kao LY, Chao AN, Wu WC, Sun MH, Su WW, et al. Retinoblastoma in Taiwan: survival and clinical characteristics. Jpn J Ophthalmol. 2021;65:546–53. DOI: https://doi.org/10.1007/s10384-021-00836-6
24. Kabre RS, Kamble KM. Retinoblastoma: a retrospective analysis of 141 patients from 1983 to 2013 at a tertiary care hospital in Nagpur, India. South Asian J Cancer. 2019;8:195–7. DOI: https://doi.org/10.4103/sajc.sajc_314_18
25. Singh L, Kashyap S. Update on pathology of retinoblastoma. Int J Ophthalmol. 2018;11:2011–6. DOI: https://doi.org/10.18240/ijo.2018.12.22
26. Gündüz AK, Mirzayev I, Temel E, Ünal E, Taçy?ld?z N, Dinçaslan H, et al. A 20-year audit of retinoblastoma treatment outcomes. Eye. 2020;34:1916–24. DOI: https://doi.org/10.1038/s41433-020-0898-9
27. Nowak MS, Romanowska-Dixon B, Grabska-Liberek I, ?urek M. Incidence and characteristics of retinoblastoma in Poland: the first nationwide study 2010–2017. Int J Environ Res Public Health. 2021;18:6539. DOI: https://doi.org/10.3390/ijerph18126539
28. Funes S, Sampor C, Villasante F, Fandiño A, Manzitti J, Sgroi M, et al. Feasibility and results of an intraarterial chemotherapy program for the conservative treatment of retinoblastoma in Argentina. Pediatr Blood Cancer. 2018;65:e27086. DOI: https://doi.org/10.1002/pbc.27086
29. Malabanan-Cabebe CGP, Santos-Gonzales MA, Te ARV, Tan RDJ, Gonzales-Sy J. Retinoblastoma in the Southern Philippines: clinical outcomes of retinoblastoma patients in a Davao tertiary hospital. Acta Med Philipp. 2024;58:45–51. DOI: https://doi.org/10.47895/amp.vi0.6754