UMMS Affiliation
Department of Ophthalmology and Visual Sciences
Publication Date
2021-04-08
Document Type
Article
Disciplines
Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Eye Diseases | Neoplasms | Ophthalmology
Abstract
Hereditary retinoblastoma survivors have substantially increased risk of subsequent malignant neoplasms (SMNs). The risk of benign neoplasms, a substantial cause of morbidity, is unclear. We calculated the cumulative incidence of developing benign tumors at 60 years following retinoblastoma diagnosis among 1128 hereditary (i.e., bilateral retinoblastoma or unilateral with family history, mutation testing was not available) and 924 nonhereditary retinoblastoma survivors diagnosed during 1914-2006 at two US medical centers with follow-up through 2016. Using Cox proportional hazards regression, we compared benign tumor risk by hereditary status and evaluated the association between benign tumors and SMNs. There were 100 benign tumors among 73 hereditary survivors (cumulative incidence = 17.6%; 95% confidence interval [CI] = 12.9-22.8%) and 22 benign tumors among 16 nonhereditary survivors (cumulative incidence = 3.9%; 95%CI = 2.2-6.4%), corresponding to 4.9-fold (95%CI = 2.8-8.4) increased risk for hereditary survivors. The cumulative incidence after hereditary retinoblastoma was highest for lipoma among males (14.0%; 95%CI = 7.7-22.1%) and leiomyoma among females (8.9%; 95%CI = 5.2-13.8%). Among hereditary survivors, having a prior SMN was associated with 3.5-fold (95%CI = 2.0-6.1) increased risk of developing a benign tumor; the reciprocal risk for developing an SMN after a benign tumor was 1.8 (95%CI = 1.1-2.9). These large-scale, long-term data demonstrate an increased risk for benign tumors after hereditary versus nonhereditary retinoblastoma. If confirmed, the association between benign tumors and SMNs among hereditary patients may have implications for long-term surveillance.
Keywords
RB1, cumulative incidence, epidemiology, hereditary retinoblastoma, leiomyoma, lipoma, retinoblastoma, retinoblastoma survivor, subsequent benign tumor, subsequent malignant neoplasms
Rights and Permissions
Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
DOI of Published Version
10.3390/cancers13081773
Source
van Hoefen Wijsard M, Schonfeld SJ, van Leeuwen FE, Moll AC, Fabius AW, Abramson DH, Seddon JM, Francis JH, Tucker MA, Kleinerman RA, Morton LM. Benign Tumors in Long-Term Survivors of Retinoblastoma. Cancers (Basel). 2021 Apr 8;13(8):1773. doi: 10.3390/cancers13081773. PMID: 33917779; PMCID: PMC8068196. Link to article on publisher's site
Journal/Book/Conference Title
Cancers
Related Resources
PubMed ID
33917779
Repository Citation
van Hoefen Wijsard M, Schonfeld SJ, van Leeuwen FE, Moll AC, Fabius AW, Abramson DH, Seddon JM, Francis JH, Tucker MA, Kleinerman RA, Morton LM. (2021). Benign Tumors in Long-Term Survivors of Retinoblastoma. Open Access Publications by UMMS Authors. https://doi.org/10.3390/cancers13081773. Retrieved from https://escholarship.umassmed.edu/oapubs/4673
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.
Included in
Congenital, Hereditary, and Neonatal Diseases and Abnormalities Commons, Eye Diseases Commons, Neoplasms Commons, Ophthalmology Commons