Next Article in Journal
Correction: Schildgen, V., et al. Human Bocavirus Infection of Permanent Cells Differentiated to Air-Liquid Interface Cultures Activates Transcription of Pathways Involved in Tumorigenesis. Cancers 2018, 10, 410
Next Article in Special Issue
Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes
Previous Article in Journal
PGC1α Loss Promotes Lung Cancer Metastasis through Epithelial-Mesenchymal Transition
Previous Article in Special Issue
Introduction of a Variant Classification System for Analysis of Genotype-Phenotype Relationships in Heritable Retinoblastoma
Article

Benign Tumors in Long-Term Survivors of Retinoblastoma

1
Department of Ophthalmology, Amsterdam UMC, Vrije Universiteit Amsterdam, Cancer Center Amsterdam, 1081 HV Amsterdam, The Netherlands
2
Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD 20892, USA
3
Department of Epidemiology, The Netherlands Cancer Institute, 1066 CX Amsterdam, The Netherlands
4
Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA
5
Department of Ophthalmology and Visual Sciences, University of Massachusetts Medical School, Worcester, MA 01605, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Simon Saule
Cancers 2021, 13(8), 1773; https://doi.org/10.3390/cancers13081773
Received: 25 February 2021 / Revised: 31 March 2021 / Accepted: 2 April 2021 / Published: 8 April 2021
It is well-established that hereditary retinoblastoma survivors have a substantially increased risk of developing subsequent malignant neoplasms (SMNs). Although clinicians have long suspected that this population is also at increased risk for developing benign neoplasms, the evidence is unclear. Benign tumors can substantially impact health status and quality of life, while raising questions for clinicians, when faced with a mass in a retinoblastoma survivor. By 60 years following retinoblastoma diagnosis, 17.6% of hereditary survivors had developed a benign tumor, with lipomas and leiomyomas being the most frequently diagnosed types. Additionally, we report both an increased risk of benign tumors after SMNs and a reciprocal increased risk of SMNs after benign tumors among hereditary retinoblastoma survivors. If confirmed, the large magnitude of the absolute risks and the association between benign tumors and SMNs in this population may have implications for long-term surveillance.
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. View Full-Text
Keywords: retinoblastoma; subsequent benign tumor; lipoma; RB1; leiomyoma; cumulative incidence; retinoblastoma survivor; epidemiology; hereditary retinoblastoma; subsequent malignant neoplasms retinoblastoma; subsequent benign tumor; lipoma; RB1; leiomyoma; cumulative incidence; retinoblastoma survivor; epidemiology; hereditary retinoblastoma; subsequent malignant neoplasms
Show Figures

Figure 1

MDPI and ACS Style

van Hoefen Wijsard, M.; Schonfeld, S.J.; van Leeuwen, F.E.; Moll, A.C.; Fabius, A.W.; Abramson, D.H.; Seddon, J.M.; Francis, J.H.; Tucker, M.A.; Kleinerman, R.A.; Morton, L.M. Benign Tumors in Long-Term Survivors of Retinoblastoma. Cancers 2021, 13, 1773. https://doi.org/10.3390/cancers13081773

AMA Style

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. 2021; 13(8):1773. https://doi.org/10.3390/cancers13081773

Chicago/Turabian Style

van Hoefen Wijsard, Milo, Sara J. Schonfeld, Flora E. van Leeuwen, Annette C. Moll, Armida W. Fabius, David H. Abramson, Johanna M. Seddon, Jasmine H. Francis, Margaret A. Tucker, Ruth A. Kleinerman, and Lindsay M. Morton. 2021. "Benign Tumors in Long-Term Survivors of Retinoblastoma" Cancers 13, no. 8: 1773. https://doi.org/10.3390/cancers13081773

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop