Trends in incidence, treatment and survival of Merkel cell carcinoma in England 2004-2018: a cohort study

Khaylen Mistry*, Nick J. Levell, Loes Hollestein, Marlies Wakkee, Tamar Nijsten, Craig S. Knott, Neil M. Steven, Paul J. Craig, Zoe C. Venables

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Scopus)


BACKGROUND: Merkel cell carcinoma (MCC) is a rare tumour with neuroendocrine differentiation and high associated mortality. Studies that describe the epidemiology of MCC are often limited by small sample size, short duration of follow-up, absence of nationwide data and paucity of data on different risk factors. OBJECTIVES: To determine the incidence, demographics and survival for MCC in England between 2004 and 2018. METHODS: This national retrospective cohort study identified all cases of MCC in England from 2004 to 2018 using national population-based data from the National Disease Registration Service. Crude counts, European age-standardized incidence rates (EASRs) and joinpoint analysis were conducted. Patient demographics and treatments received were described. Multivariable Cox regression analysis was used to study risk factors for MCC-specific mortality, by including a priori defined demographic factors, tumour characteristics and immunosuppression. Treatment data were not included in the Cox regression analysis. RESULTS: A total of 3775 MCC tumours were registered. The median age at diagnosis was 81 years (interquartile range 74-87). Overall, 96·6% of patients identified as White ethnicity, and 8·3% of patients were immunosuppressed. The most common site was the face (27·4%). Patients most often presented with stage one disease (22·8%); however, stage was unknown in 31·0%. In total, 80·7% of patients underwent surgical excision, 43·5% radiotherapy and 9·2% systemic therapy. The EASR increased from 0·43 per 100 000 person-years (PYs) to 0·65 per 100 000 person-years between 2004 and 2018, representing a significant annual percentage change of 3·9%. The EASR was greater in men than in women for all years, with an overall male-to-female ratio of 1·41 : 1. The highest EASR was in South West England. Five-year disease-specific survival was 65·6% [95% confidence interval (CI) 63·8-67·4], with a median follow-up of 767 days. MCC-specific mortality increased with age [hazard ratio (HR) 1·02, 95% CI 1·02-1·03], deprivation (HR 1·43, 95% CI 1·16-1·76), immunosuppression (HR 2·80, 95% CI 2·34-3·34) and stage at diagnosis (HR 8·24, 95% CI 5·84-11·6). CONCLUSIONS: This study presents the largest national MCC dataset in Europe, and the most complete reporting of MCC incidence and survival ever published. With the EASR of MCC increasing and high associated mortality, this study encourages further research into the pathology, diagnosis and therapeutic options for MCC to support management guidelines.

Original languageEnglish
Pages (from-to)228-236
Number of pages9
JournalThe British journal of dermatology
Issue number2
Publication statusPublished - Feb 2023

Bibliographical note

Funding Information:
Conflicts of interest: N.M.S. has received research grants from the Medical Research Council Efficacy and Mechanism Evaluation fund. He has also received payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Merck and Pfizer. He also participates on a data safety monitoring board and advisory board for Combi-I (Novartis). N.J.L. is a trustee of the British Association of Dermatologists. All of the other authors have no conflicts of interest to disclose.

Publisher Copyright:
© 2022 The Author(s). Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved.


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