Abstract
Background/objectives
Early diagnosis, care and treatment of retinoblastoma is a challengeable issue for Iranian health system. This study was designed and conducted in a referral multidisciplinary centre in the capital city of Iran to evaluate management, care, prognosis and survival rates of paediatric patients with retinoblastoma.
Methods
In this retrospective study, a total number of 309 patients younger than 15 years, diagnosed with retinoblastoma, who referred for diagnosis and treatment to MAHAK’s Pediatric Cancer Treatment and Research Center (MPCTRC) from 2007 to 2017 were evaluated. All data were analyzed via SPSS version 22 software in regard of parametric and non-parametric data. Survival rates were analyzed using the Kaplan–Meyer method.
Results
The mean age of patients was 20 months and the majority of patients (77%) had leukocoria as a common clinical symptom at the time of diagnosis. Primary treatment methods were systemic chemotherapy (94%), laser (35%) and primary enucleation (28%). Relapses occurred in nearly 42% of cases, and the median time from diagnosis to the first relapse was 9 months. At the time analyzing the data, 11% of patients died. Patients’ 5-year OS and RFS rates were 79.6% and 41.5%, respectively.
Conclusion
Comparing results with other conducted studies identifies that the recurrence rate was high in our considered patients. Also, OS and RFS rates in our study were not as considerable as other reports. Screening methods, updating protocols and follow-up of patients may lead to improvements in survival rates of patients with retinoblastoma.
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References
Dome JSR-GC, Spunt SL, Santana VM. Chapter 95: Pediatric solid tumors. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, editors. Abeloff’s clinical oncology. 5th ed. Philadelphia: Elsevier; 2014.
Hurwitz RL, Shields CL, Shields JA. Retinoblastoma. In: Pizzo PA, Poplack DG, editors. Principles and practice of pediatric oncology. Philadelphia: Lippincott Williams & Wilkins; 2016.
Dimaras H, Corson TW, Cobrinik D, White A, Zhao J, Munier FL, et al. Retinoblastoma. Nat Rev Dis Prim. 2015;1:15021.
Naseripour M, Ghasemi FK, Bakhtiari P, Vosoughi P, Arian F. Retinoblastoma survival in Iran: 10 years experience of a referral center. Iranian Journal of Ophtalmology. 2009;21:17–24.
Nyawira G, Kahaki K, Kariuki-Wanyoike M, Survival among retinoblastoma patients at the Kenyatta National Hospital, Kenya. JOECSA. 2013;17:15–19.
Dean M, Bendfeldt G, Lou H, Giron V, Garrido C, Valverde P, et al. Increased incidence and disparity of diagnosis of retinoblastoma patients in Guatemala. Cancer Lett. 2014;351:59–63.
Group TWB. The World Bank 2019. https://data.worldbank.org/?locations=XT-IR.
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;93:28–32.
Abramson DH, Dunkel IJ, Brodie SE, Kim JW, Gobin YP. A phase I/II study of direct intraarterial (ophthalmic artery) chemotherapy with melphalan for intraocular retinoblastoma: initial results. Ophthalmology. 2008;115:1398–404. e1.
Munier FL, Gaillard M-C, Balmer A, Soliman S, Podilsky G, Moulin AP, et al. Intravitreal chemotherapy for vitreous disease in retinoblastoma revisited: from prohibition to conditional indications. Br J Ophthalmol. 2012;96:1078–83.
Linn AM. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin N Am. 2005;18:41–53. viii
Rowlands MA, Mondesire-Crump I, Levin A, Mauguen A, Francis JH, Dunkel IJ, et al. Total retinal detachments due to retinoblastoma: outcomes following intra-arterial chemotherapy/ophthalmic artery chemosurgery. PloS ONE 2018;13:e0195395.
Nabie R, Taheri N, Fard AM, Fouladi RF. Characteristics and clinical presentations of pediatric retinoblastoma in North-western Iran. Int J Ophthalmol. 2012;5:510.
Berry JL, Kogachi K, Aziz HA, McGovern K, Zolfaghari E, Murphree AL, et al. Risk of metastasis and orbital recurrence in advanced retinoblastoma eyes treated with systemic chemoreduction versus primary enucleation. Pediatr Blood Cancer. 2017;64:e26270.
Berry JL, Jubran R, Kim JW, Wong K, Bababeygy SR, Almarzouki H, et al. Long‐term outcomes of Group D eyes in bilateral retinoblastoma patients treated with chemoreduction and low‐dose IMRT salvage. Pediatr Blood Cancer. 2013;60:688–93.
Brennan RC, Qaddoumi I, Mao S, Wu J, Billups CA, Stewart CF, et al. Ocular salvage and vision preservation using a topotecan-based regimen for advanced intraocular retinoblastoma. J Clin Oncol. 2017;35:72–7.
Chen Q, Zhang B, Dong Y, Mo X, Zhang L, Huang W, et al. Comparison between intravenous chemotherapy and intra-arterial chemotherapy for retinoblastoma: a meta-analysis. BMC Cancer 2018;18:486.
Shields CL, Bianciotto CG, Jabbour P, Griffin GC, Ramasubramanian A, Rosenwasser R, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 2, treatment complications. Arch Ophthalmol. 2011;129:1407–15.
Kaliki S, Shields CL, Shah SU, Eagle RC, Shields JA, Leahey A. Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma. Arch Ophthalmol. 2011;129:1422–7.
Berry JL, Shah S, Bechtold M, Zolfaghari E, Jubran R, Kim JW. Long‐term outcomes of Group D retinoblastoma eyes during the intravitreal melphalan era. Pediatr Blood Cancer. 2017;64:e26696.
Munier FL, Mosimann P, Puccinelli F, Gaillard M-C, Stathopoulos C, Houghton S, et al. First-line intra-arterial versus intravenous chemotherapy in unilateral sporadic group D retinoblastoma: evidence of better visual outcomes, ocular survival and shorter time to success with intra-arterial delivery from retrospective review of 20 years of treatment. Br J Ophthalmol. 2017;101:1086–93.
Shields CL, Bianciotto CG, Jabbour P, Ramasubramanian A, Lally SE, Griffin GC, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 1, control of retinal tumors, subretinal seeds, and vitreous seeds. Arch Ophthalmol. 2011;129:1399–406.
Chen M, Jiang H, Zhang J, Shen G, Jiang Y, Li H, et al. Outcome of intra‐arterial chemotherapy for retinoblastoma and its influencing factors: a retrospective study. Acta Ophthalmol. 2017;95:613–8.
Tuncer S, Sencer S, Kebudi R, Tanyıldız B, Cebeci Z, Aydın K. Superselective intra‐arterial chemotherapy in the primary management of advanced intra‐ocular retinoblastoma: first 4‐year experience from a single institution in Turkey. Acta Ophthalmol. 2016;94:e644–e51.
Shields CL, Shields JA. Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Curr Opin Ophthalmol. 2010;21:203–12.
Friedman DL, Krailo M, Villaluna D, Gombos D, Langholz B, Jubran R, et al. Systemic neoadjuvant chemotherapy for group B intraocular retinoblastoma (ARET0331): a report from the Children’s oncology group. Pediatr Blood Cancer. 2017;64:e26394.
Thampi S, Hetts SW, Cooke DL, Stewart PJ, Robbins E, Banerjee A, et al. Superselective intra-arterial melphalan therapy for newly diagnosed and refractory retinoblastoma: results from a single institution. Clin Ophthalmol. 2013;7:981.
Shields CL, Manjandavida FP, Lally SE, Pieretti G, Arepalli SA, Caywood EH, et al. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma. Ophthalmology. 2014;121:1453–60.
Fabian ID, Stacey AW, Johnson KP, Onadim Z, Chowdhury T, Duncan C, et al. Primary intravenous chemotherapy for group D retinoblastoma: a 13-year retrospective analysis. Br J Ophthalmol. 2017;101:82–8.
Yousef YA, Nazzal RM, Khalil MB, Deebajah R, Mehyar M, Hajja S, et al. Management outcome (s) in eyes with retinoblastoma previously inadequately treated with systemic chemotherapy alone without focal therapy. Oman J Ophthalmol. 2017;10:70.
Naseripour M. “Retinoblastoma survival disparity”: the expanding horizon in developing countries. Saudi J Ophthalmol. 2012;26:157–61.
Ghassemi F, Rahmanikhah E, Roohipoor R, Karkhaneh R, Faegh A. Regression patterns in treated retinoblastoma with chemotherapy plus focal adjuvant therapy. Pediatr Blood Cancer. 2013;60:599–604.
Ghassemi F, Ghanaati H, Karkhaneh R, Boujabadi L, Tabatabaie SZ, Rajabi MT. Outcome of retinoblastoma following limited sessions of intra-arterial chemotherapy in Iran. Iran J Radiol. 2014;11:e16958.
Canturk S, Qaddoumi I, Khetan V, Ma Z, Furmanchuk A, Antoneli C, et al. Survival of retinoblastoma in less-developed countries impact of socioeconomic and health-related indicators. Br J Ophthalmol. 2010;94:1432–6.
Acknowledgements
Authors are thankful for their missed colleague, Dr Elham Olad, who gathered data of this study. God bless her. Also, authors appreciate Dr Khubbin Khoshnazar who entered data in SPSS.
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Faranoush, M., Mehrvar, N., Tashvighi, M. et al. Retinoblastoma presentation, treatment and outcome in a large referral centre in Tehran: a 10-year retrospective analysis. Eye 35, 575–583 (2021). https://doi.org/10.1038/s41433-020-0907-z
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DOI: https://doi.org/10.1038/s41433-020-0907-z
