Abstract

Background: Retinoblastoma is the most common primary intraocular malignancy of childhood. Despite high survival and eye salvage as the result of various types of therapies, retinoblastoma remains a disease that places a considerable burden on developing countries. Our study attempted to analyse the research trends in retinoblastoma research and compare contributions from different countries, institutions, journals, and authors.Methods: We extracted all publications concerning retinoblastoma from 2001 to 2021 from the Web of Science database. Microsoft Excel and VOSviewer were employed to collect publication data, analyse publication trends, and visualize relevant results.Results: A total of 1,675 publications with 30,148 citations were identified. The United States contributed the most publications (643) and citations (16,931 times) with the highest H-index value (67) as of February 4, 2021. China ranked second in the number of publications (259), while ranking fourth in both citations (2,632 times) and the H-index (26) ranked fourth. The British Journal of Ophthalmology was the most productive journal concerning retinoblastoma, and Abramson DH had published the most papers in the field. Keywords were categorized into three clusters; tumor-related research, clinical research, and management-related research. The keywords “intravitreal,” “intraarterial,” and “intravenous” appeared the most frequently, with the average appearing year being 2018.1, 2017.7, and 2017.1, respectively. Management-related research has been recognized as a heavily researched topic in the field.Conclusion: We conclude that the United States, China, and India made the most exceptional contributions in the field of retinoblastoma research, while China still has a disparity between the quantity and quality of publications. Management-related research, including intravitreal, intraarterial, and intravenous chemotherapy was considered as a potential focus for future research.

Highlights

  • Retinoblastoma, the most common primary intraocular malignancy of childhood, is initiated by mutation of both RB1 alleles in a single susceptible developing retinal cell, undergoes the limited proliferation of an RB1−/− retinal cell to form a non-malignant retinal tumor and experiences uncontrolled proliferation and malignant transformation based on genetic or epigenetic alterations [1].The incidence of retinoblastoma is constant without race or gender differences at one case every 15,000 to 20,000 live births worldwide [2]

  • Based on the fact that the Science Citation Index-Expanded (SCIE) of Web of Science (WOS) was considered the optimal database for bibliometric analysis, a literature search was conducted for the years 2001 to 2021 using the WOS database

  • The two-hit hypothesis was initially proposed by Alfred Knudson, an American physician and cancer geneticist, in 1971 [26], which shifted the concept of the cause of cancer from oncogene activation to tumor-suppressor loss of function, indicating that American scholars took the lead in comprehensively understanding the pathogenesis of retinoblastoma and developing early genetic testing and screening programmes

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Summary

Introduction

Retinoblastoma, the most common primary intraocular malignancy of childhood, is initiated by mutation of both RB1 alleles in a single susceptible developing retinal cell, undergoes the limited proliferation of an RB1−/− retinal cell to form a non-malignant retinal tumor and experiences uncontrolled proliferation and malignant transformation based on genetic or epigenetic alterations [1].The incidence of retinoblastoma is constant without race or gender differences at one case every 15,000 to 20,000 live births worldwide [2]. Asia and Africa, which experience large populations and high birth rates, bear the greatest burden of retinoblastoma [3]. They carry the highest mortality of 40 to 70%, compared with 3 to 5% in Europe, Canada, and the USA, which contributes to the delay in diagnosis and the lack of access to canonical treatment [4,5,6]. Leukocoria is the most common clinical feature of retinoblastoma, in which the normal pupillary is replaced by a whitish discoloration as a result of abnormal growth and calcification of the developing intraocular tumor, followed by strabismus, owing to loss of central vision from the growing tumor. Our study attempted to analyse the research trends in retinoblastoma research and compare contributions from different countries, institutions, journals, and authors

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