Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Article
  • Published:

Incidence and survival of uveal melanoma in Northern Ireland: how incomplete data can skew results in rare cancers

Eye volume 37, pages 2454–2460 (2023)Cite this article

Subjects

Abstract

Background

The majority of Northern Irish uveal melanoma (UM) patients are diagnosed in Sheffield. This study aims to present incidence and survival outcomes for UM patients from Northern Ireland (NI).

Methods

Collaborative retrospective study between Sheffield and Northern Ireland Cancer Registry (NICR). For UM cases not on both databases, outcomes and survival rates (via Kaplan-Meier analysis) were compared. Anonymised NICR data were used to calculate whole-population incidence of UM for NI.

Results

In total, 161 patients from NI were diagnosed in Sheffield, 90 of which were not registered with NICR at the start of this study. Data-omissions were not consistent across patient groups, leading to significant differences between those patients registered and those not. Registered patients had an all-cause 5-year survival rate of only 68.9% compared to 92.5% of those not registered (p < 0.01) and were >17x more likely to have systemic metastases than those not registered (p < 0·001). Following rectification of data-omissions, the European age-standardised incidence rate of UM for NI was 8·6 per million.

Conclusions

This study illustrates the impact of incomplete population-wide data, serving as a real-world lesson in case-identification bias. Rare cancers are at higher risk of omission due to systemic failures as the small numbers involved are not detected by system-wide validation procedures. Following this study, data-transfer agreements between England and NI were actioned, preventing future data-omissions. We present survival and incidence data for UM in NI for the first time, showing the incidence is amongst the highest in Europe, with good survival rates.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Fig. 1
Fig. 2: Venn diagrams illustrating the groups described in this study, and the number of cases in each group.
Fig. 3

Similar content being viewed by others

Data availability

Northern Ireland Cancer Registry data are available and can be accessed by direct application. The data for the Sheffield cohort can be accessed by written application to the authors.

References

  1. Virgili G, Gatta G, Ciccolallo L, Capocaccia R, Biggeri A, Crocetti E, et al. Incidence of uveal melanoma in Europe. Ophthalmology. 2007;114:2309–15.

    Article  PubMed  Google Scholar 

  2. Baily C, O’Neill V, Dunne M, Cunningham M, Gullo G, Kennedy S, et al. Uveal melanoma in Ireland. Ocul Oncol Pathol. 2019;5:195–204. https://doi.org/10.1159/000492391.

    Article  PubMed  Google Scholar 

  3. Singh AD, Topham A. Survival rates with uveal melanoma in the United States: 1973–1997. Ophthalmology. 2003;110:962–5. https://doi.org/10.1016/S0161-6420(03)00077-0.

    Article  PubMed  Google Scholar 

  4. Virgili G, Gatta G, Ciccolallo L, Capocaccia R, Biggeri A, Crocetti E, et al. Survival in patients with uveal melanoma in Europe. Archives of ophthalmology, 2008. 126. pp. 1413-8. ISSN 0003-9950 https://doi.org/10.1001/archopht.126.10.1413

  5. Beral V, Peto R. UK cancer survival statistics. BMJ. 2010;341:c4112 https://doi.org/10.1136/bmj.c4112

    Article  PubMed  Google Scholar 

  6. Kearney TM, Donnelly C, Kelly JM, O’Callaghan EP, Fox CR, Gavin AT Validation of the completeness and accuracy of the Northern Ireland Cancer Registry. Cancer Epidemiol. 2015;39:401-4. https://doi.org/10.1016/j.canep.2015.02.005.

  7. AJCC Cancer Staging Manual 8th ed. 2017, Corr. 3rd printing 2018 Edition

  8. Nathan P, Cohen V, Coupland S, Curtis K, Damato B, Evans J, et al. Uveal melanoma UK national guidelines. Eur J Cancer. 2015;51:2404–12. https://doi.org/10.1016/j.ejca.2015.07.013. Epub 2015 Aug 13. PMID: 26278648

    Article  CAS  PubMed  Google Scholar 

  9. International classification of diseases for oncology (ICD-O), 3rd ed., 1st revision3rd edition, 1st revision, https://apps.who.int/iris/handle/10665/96612

  10. Stang A, Parkin DM, Ferlay J, Jöckel KH. International uveal melanoma incidence trends in view of a decreasing proportion of morphological verification. Int J Cancer. 2005;114:114–23. https://doi.org/10.1002/ijc.20690. PMID: 15523698

    Article  CAS  PubMed  Google Scholar 

  11. Vajdic CM, Kricker A, Giblin M, McKenzie J, Aitken J, Giles GG, et al. Incidence of ocular melanoma in Australia from 1990 to 1998. Int J Cancer. 2003;105:117–22. https://doi.org/10.1002/ijc.11057. PMID: 12672041

    Article  CAS  PubMed  Google Scholar 

  12. Iscovich J, Ackerman C, Andreev H, Pe’er J, Steinitz R. An epidemiological study of posterior uveal melanoma in Israel, 1961-1989. Int J Cancer. 1995;61:291–5. https://doi.org/10.1002/ijc.2910610302. 341 PMID: 7729936

    Article  CAS  PubMed  Google Scholar 

  13. Kaliki S, Shields CL. Uveal melanoma: relatively rare but deadly cancer. Eye. 2017;31:241–57. https://doi.org/10.1038/eye.2016.275

    Article  CAS  PubMed  Google Scholar 

  14. Gilbert E, O’Reilly S, Merrigan M, McGettigan D, Molloy AM, Brody LC, et al. The Irish DNA atlas: revealing fine-scale population structure and history within Ireland. Sci Rep. 2017;7:17199. https://doi.org/10.1038/s41598-017-17124-4. Erratum in: Sci Rep. 2018 May 3;8(1):7208.

  15. Aronow ME, Topham AK, Singh AD. Uveal melanoma: 5-year update on incidence, treatment, and survival (SEER 1973-2013). Ocul Oncol Pathol. 2018;4:145–51. https://doi.org/10.1159/000480640

    Article  PubMed  Google Scholar 

  16. Garg G, Finger PT, Kivelä TT, Simpson ER, Gallie BL, Saakyan S, et al. Patients presenting with metastases: stage IV uveal melanoma, an international study. Br J Ophthalmol Published Online First: 15 January 2021. https://doi.org/10.1136/bjophthalmol-2020-317949

  17. Steckler AM, Francis JH, Shoushtari AN, Abramson DH, Barker CA. Uveal melanoma metastatic at initial diagnosis: a case series. Melanoma Res. 2022;32:120–3. https://doi.org/10.1097/CMR.0000000000000807.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Rantala ES, Hernberg MM, Piperno-Neumann S, Grossniklaus HE, Kivelä TT Metastatic uveal melanoma: The final frontier. Prog Retin Eye Res. 2022:101041. https://doi.org/10.1016/j.preteyeres.2022.101041.

  19. Robinson D, Sankila R, Hakulinen T, Møller H. Interpreting international comparisons of cancer survival: the effects of incomplete registration and the presence of death certificate only cases on survival estimates. Eur J Cancer. 2007;43:909–13. https://doi.org/10.1016/j.ejca.2007.01.007.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

This research has been conducted using data from the Northern Ireland Cancer Registry (NICR) which is funded by the Public Health Agency, Northern Ireland. However, the interpretation and conclusions of the data are the sole responsibility of the author(s). The author(s) acknowledge the contribution of the NICR staff in the production of the NICR data. Like all Cancer Registries our work uses data provided by patients and collected by the health service as part of their care and support.

Author information

Authors and Affiliations

  1. Sheffield Ocular Oncology Service, Royal Hallamshire Hospital, Sheffield, UK

    Hibba Quhill, Ian G. Rennie, Sachin M. Salvi & Paul Rundle

  2. Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, UK

    Joanna M. Jefferis

  3. Northern Ireland Cancer Registry, Queen’s University Belfast, Belfast, Northern Ireland, UK

    Anna Gavin, Deirdre Fitzpatrick & Gerard Savage

  4. Department of Ophthalmology, Royal Victoria Hospital, Belfast, Northern Ireland, UK

    David Curragh

Authors
  1. Hibba Quhill
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Joanna M. Jefferis
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ian G. Rennie
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sachin M. Salvi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Anna Gavin
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Deirdre Fitzpatrick
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Gerard Savage
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. David Curragh
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Paul Rundle
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

HQ conceptualised the study, performed the literature search, study design, data collection, data analysis, data interpretation, produced the figures and wrote the original draft of the paper. JMJ contributed to data analysis, interpretation, and verification of the underlying data, produced the figures, and reviewed and edited the paper. IGR, AG and PR contributed to conceptualisation and methodology and reviewed and edited the paper. DF and GS contributed to data collection, data analysis, verification of the underlying data and edited the paper. SS and DC contributed to the review and editing of the paper.

Corresponding author

Correspondence to Hibba Quhill.

Ethics declarations

Competing interests

The authors declare no competing interests.

Ethics Approval and Consent to Participate

Ethical approval for the NICR databases (including the waiving of requirement for individual patient consent) and data analysis has been granted by the Office for Research Ethics Committees of Northern Ireland (ORECNI reference 15/NI/0203), recently renewed in October 2020 (REC REF 20-NI- 0132). This study complies with the declaration of Helsinki.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary information

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Quhill, H., Jefferis, J.M., Rennie, I.G. et al. Incidence and survival of uveal melanoma in Northern Ireland: how incomplete data can skew results in rare cancers. Eye 37, 2454–2460 (2023). https://doi.org/10.1038/s41433-022-02352-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/s41433-022-02352-4

Search

Advanced search

Quick links