Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
BMC Pediatrics
Published in: BMC Pediatrics 1/2023

Open Access 01-12-2023 | Retinoblastoma | Research

Clinical characteristics, treatment and prognosis of infants with retinoblastoma: a multicenter, 10-year retrospective analysis

Authors: Yi Zhang, Yizhuo Wang, Tian Zhi, Mei Jin, Dongsheng Huang, Xiaoli Ma

Published in: BMC Pediatrics | Issue 1/2023

Login to get access

Abstract

Background

To summarize the characteristics and treatment, and analyze the prognosis of large number of infants with retinoblastoma (RB) in China through a multicenter, 10-year retrospective analysis.

Methods

The data of RB infants were collected from multiple centers. The characteristics and survival prognosis were analyzed. The overall survival (OS) rate was estimated by the Kaplan–Meier method. Multivariate Cox survival analysis was to evaluate the independent risk factors affecting the prognosis of RB infants.

Results

A total of 373 RB infants (202 boys and 171 girls) were included, the median age was 6.22 months (10 days to 11.93 months). The median follow-up time of RB infants was 18.4 (1.02–122.81 months). After excluding the lost to follow-up cases, the OS rate was 97.7% (345/353). Kaplan–Meier survival analysis indicated that 9 cases died and the median survival time was not reached. Univariate analysis of prognostic factors revealed eye affected, presenting signs, left eye stage and recurrence to be poor prognostic factors for OS rate in RB infants (all P < 0.05). Multivariate Cox regression analyses for OS showed recurrence (HR = 1.376, 95% CI: 0.878–2.156, P = 0.048) was an independent factor for prognosis of infants with RB. The median survival time of infants underwent chemotherapy + intra-arterial chemotherapy (IAC) + enucleation + vitrectomy was the longest than other treatments (n = 9, 47.64 months, OS = 100%, all P < 0.05). There was a history of RB in 17 infants' lineal relatives. Kaplan-merier survival analysis indicated 1 case died and the median survival time was not reached.

Conclusion

Recurrence is an independent factor for prognosis of RB infants, which still needs attention after treatment. Early screening, comprehensive treatments and follow-up of patients may lead to improvements of prognosis of RB infants.
Literature
1.
Dimaras H, Corson TW. Retinoblastoma, the visible CNS tumor: A review. J Neurosci Res. 2019;97(1):29–44.CrossRefPubMed
2.
3.
Jain M, Rojanaporn D, Chawla B, Sundar G, Gopal L, Khetan V. Retinoblastoma in Asia. Eye. 2019;33(1):87–96.CrossRefPubMed
4.
Fang X, Wang Y, Yin J, Guo Y, Jia L, Zhang C, Jin M, Ni X, Zhao J. Clinical features and survival of Chinese children with trilateral retinoblastoma during 2006–2019: a retrospective multicenter study. Am J Ophthalmol. 2021;223:184–92.CrossRefPubMed
5.
Mendoza PR, Grossniklaus HE. The Biology of Retinoblastoma. Prog Mol Biol Transl Sci. 2015;134:503–16.CrossRefPubMed
6.
AlAli A, Kletke S, Gallie B, Lam WC. Retinoblastoma for pediatric ophthalmologists. Asia-Pacific journal of ophthalmology. 2018;7(3):160–8.PubMed
7.
Roy SR, Kaliki S. Retinoblastoma: a major review. Mymensingh Med J. 2021;30(3):881–95.PubMed
8.
Ueda T, Koga Y, Yoshikawa H, Tanabe M, Yamana K, Oba U, Nakashima K, Ono H, Ichimura T, Hasegawa S, et al. Survival and ocular preservation in a long-term cohort of Japanese patients with retinoblastoma. BMC Pediatr. 2020;20(1):37.CrossRefPubMedPubMedCentral
9.
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):1372–5.CrossRefPubMed
10.
Skalet AH, Gombos DS, Gallie BL, Kim JW, Shields CL, Marr BP, Plon SE, Chevez-Barrios P. Screening children at risk for retinoblastoma: consensus report from the American association of ophthalmic oncologists and pathologists. Ophthalmology. 2018;125(3):453–8.CrossRefPubMed
11.
Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodriguez-Galindo C, Schvartzman E, et al. A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer. 2006;47(6):801–5.CrossRefPubMed
12.
Shields CL, Shields JA. Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophthalmol. 2006;17(3):228–34.CrossRefPubMed
13.
Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA. The international classification of retinoblastoma predicts chemoreduction success. Ophthalmology. 2006;113(12):2276–80.CrossRefPubMed
14.
Wilson MW, Rodriguez-Galindo C, Haik BG, Moshfeghi DM, Merchant TE, Pratt CB. Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma. Ophthalmology. 2001;108(11):2106–14 (discussion 2114-2105).CrossRefPubMed
15.
Shields CL, Fulco EM, Arias JD, Alarcon C, Pellegrini M, Rishi P, Kaliki S, Bianciotto CG, Shields JA. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye. 2013;27(2):253–64.CrossRefPubMed
16.
Francis JH, Marr BP, Brodie SE, Abramson DH. Anterior Ocular Toxicity of Intravitreous Melphalan for Retinoblastoma. JAMA Ophthalmol. 2015;133(12):1459–63.CrossRefPubMedPubMedCentral
17.
Fabian ID, Onadim Z, Karaa E, Duncan C, Chowdhury T, Scheimberg I, Ohnuma SI, Reddy MA, Sagoo MS. The management of retinoblastoma. Oncogene. 2018;37(12):1551–60.CrossRefPubMed
18.
Mirzayev I, Gunduz AK, Bicer O, Tarlan B. The final diagnosis: retinoblastoma or Pseudoretinoblastoma. J Pediatr Ophthalmol Strabismus. 2021;58(3):161–7.CrossRefPubMed
19.
20.
Shields CL, Schoenberg E, Kocher K, Shukla SY, Kaliki S, Shields JA. Lesions simulating retinoblastoma (pseudoretinoblastoma) in 604 cases: results based on age at presentation. Ophthalmology. 2013;120(2):311–6.CrossRefPubMed
21.
Temming P, Viehmann A, Biewald E, Lohmann DR. Sporadic unilateral retinoblastoma or first sign of bilateral disease? Br J Ophthalmol. 2013;97(4):475–80.CrossRefPubMed
22.
Parrilla-Vallejo M, Perea-Perez R, Relimpio-Lopez I, Montero-de-Espinosa I, Rodriguez-de-la-Rua E, Terron-Leon JA, Diaz-Granda MJ, Coca-Gutierrez L, Ponte-Zuniga B, Espejo-Arjona F. Retinoblastoma: The importance of early diagnosis. Arch de la Sociedad Espanola de Oftalmol. 2018;93(9):423–30.CrossRef
23.
24.
Global Retinoblastoma Study G, Fabian ID, Abdallah E, Abdullahi SU, Abdulqader RA, Adamou Boubacar S, Ademola-Popoola DS, Adio A, Afshar AR, Aggarwal P, et al. Global Retinoblastoma Presentation and Analysis by National Income Level. JAMA oncology. 2020;6(5):685–95.CrossRef
25.
Huang D, Zhang Y, Zhang W, Wang Y, Zhang P, Hong L, Zhou Y, Han T, Zhi T. Study on clinical therapeutic effect including symptoms, eye preservation rate, and follow-up of 684 children with retinoblastoma. Eur J Ophthalmol. 2013;23(4):532–8.CrossRefPubMed
26.
Pant G, Verma N, Kumar A, Pooniya V, Gupta SK. Outcome of extraocular retinoblastoma in a resource limited center from low middle income country. Pediatr Hematol Oncol. 2017;34(8):419–24.CrossRefPubMed
27.
Palioura S, Gobin YP, Brodie SE, Marr BP, Dunkel IJ, Abramson DH. Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment. Pediatr Blood Cancer. 2012;59(5):859–64.CrossRefPubMed
28.
Mirzayev I, Gunduz AK, Yavuz K, Sekkeli MZ, OzalpAtes FS, Unal E, Tacyildiz N. Secondary intra-arterial chemotherapy and/or intravitreal chemotherapy as salvage treatment for retinoblastoma. Eur J Ophthalmol. 2021;31(5):2692–8.CrossRefPubMed
29.
Francis JH, Roosipu N, Levin AM, Brodie SE, Dunkel IJ, Gobin YP, Abramson DH. Current Treatment of Bilateral Retinoblastoma: The Impact of Intraarterial and Intravitreous Chemotherapy. Neoplasia. 2018;20(8):757–63.CrossRefPubMedPubMedCentral
30.
Zhang Y, Wang Y, Huang D, Ma J, Zhang W, Gu H, Zhou Y, Yi Y, Zhang P. Correlation between Family RB1 gene pathogenic variant with clinical features and prognosis of retinoblastoma under 5 years old. Dis Markers. 2021;2021:9981028.PubMedPubMedCentral
31.
Goncalves LF, Ramasubramanian A, Grebe T, Riemann M, Moncrief D, Cornejo P. Prenatal diagnosis of bilateral retinoblastomas by multimodality fetal imaging: case report and review of the literature. Clin Imaging. 2021;78:121–6.CrossRefPubMed
32.
Dimaras H, Khetan V, Halliday W, Orlic M, Prigoda NL, Piovesan B, Marrano P, Corson TW, Eagle RC Jr, Squire JA, et al. Loss of RB1 induces non-proliferative retinoma: increasing genomic instability correlates with progression to retinoblastoma. Hum Mol Genet. 2008;17(10):1363–72.CrossRefPubMed
Metadata
Title
Clinical characteristics, treatment and prognosis of infants with retinoblastoma: a multicenter, 10-year retrospective analysis
Authors
Yi Zhang
Yizhuo Wang
Tian Zhi
Mei Jin
Dongsheng Huang
Xiaoli Ma
Publication date
01-12-2023
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2023
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/s12887-023-03984-5

Other articles of this Issue 1/2023

BMC Pediatrics 1/2023 Go to the issue

Research

Early echocardiographic pulmonary artery measurements as prognostic indicators in left congenital diaphragmatic hernia

Research

The development and productivity of a measure for identifying low language abilities in children aged 24–36 months

Research

Impairments in fine motor skills in children with Acute Lymphoblastic Leukaemia. A cross-sectional study

Research

Health beliefs on type 2 diabetes: a methodological research for development and psychometric evaluation of “DIABA” (Diabetes-related Instrument to Assess Beliefs of Adolescents) health beliefs on type 2 diabetes

Research

The burden of childhood and adolescent cancers in North Africa and the Middle East (NAME) region: findings from the Global Burden of Disease study 2019

Case Report

Multiple vascular anomalies and refractory pericardial effusion in a young patient with Cantu syndrome: a case report and review of the literature