Benign tumors in long-term survivors of retinoblastoma Journal Article


Authors: 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.
Article Title: Benign tumors in long-term survivors of retinoblastoma
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 non-hereditary 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. © 2021 by the authors. Licensee MDPI, Basel, Switzerland.
Keywords: retinoblastoma; epidemiology; cumulative incidence; lipoma; leiomyoma; hereditary retinoblastoma; rb1; retinoblastoma survivor; subsequent benign tumor; subsequent malignant neoplasms
Journal Title: Cancers
Volume: 13
Issue: 8
ISSN: 2072-6694
Publisher: MDPI  
Date Published: 2021-04-01
Start Page: 1773
Language: English
DOI: 10.3390/cancers13081773
PROVIDER: scopus
PMCID: PMC8068196
PUBMED: 33917779
DOI/URL:
Notes: Article -- Export Date: 3 May 2021 -- Source: Scopus
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  1. Jasmine Helen Francis
    257 Francis
  2. David H Abramson
    390 Abramson