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Original Investigation |

Incidence and Survival for Merkel Cell Carcinoma in Queensland, Australia, 1993-2010

Danny R. Youlden, BSc1; H. Peter Soyer, MD, FACD2; Philippa H. Youl, MPH, PhD1; Lin Fritschi, MBBS, PhD3; Peter D. Baade, PhD1
[+] Author Affiliations
1Cancer Council Queensland, Brisbane, Queensland, Australia
2Dermatology Research Centre, The University of Queensland, School of Medicine, Translational Research Institute, Princess Alexandra Hospital, Brisbane, Queensland, Australia
3Western Australian Institute for Medical Research, The University of Western Australia, Perth, Western Australia, Australia
JAMA Dermatol. 2014;150(8):864-872. doi:10.1001/jamadermatol.2014.124.
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Importance  Merkel cell carcinoma (MCC) is an uncommon but highly invasive form of skin cancer. The mechanisms that cause MCC are yet to be fully determined.

Objectives  To compare the incidence and survival rates of MCC in Queensland, Australia, known to be a high-risk area, with MCC incidence and survival elsewhere in the world. We also analyzed incidence trends and differences in survival by key demographic and clinical characteristics.

Design, Setting, and Participants  Retrospective cohort study of population-based administrative data for MCC collected by the Queensland Cancer Registry and supplemented with detailed histopathologic data. Deidentified records were obtained of all Queensland residents diagnosed as having MCC during the period from 1993 to 2010. A subsample of histopathologic records were reviewed by a senior dermatopathologist to determine the potential for misclassification. A total of 879 eligible cases of MCC were included in the study.

Main Outcomes and Measures  Incidence rates were directly age standardized to the 2000 United States Standard Population. Trends were examined using Joinpoint software with results expressed in terms of the annual percentage change. The period method was used to calculate 5-year relative survival, and adjusted hazard ratios were obtained from multivariate Poisson models.

Results  There were 340 cases of MCC diagnosed in Queensland between 2006 and 2010, corresponding to an incidence rate of 1.6 per 100 000 population. Men (2.5 per 100 000) had higher incidence than women (0.9 per 100 000), and rates peaked at 20.7 per 100 000 for persons 80 years or older. The overall incidence of MCC increased by an average of 2.6% per year from 1993 onwards. Relative survival was 41% after 5 years, with significantly better survival found for those younger than 70 years at diagnosis (56%-60%), those with tumors on the face or ears (51%), and those with stage I lesions (49%).

Conclusions and Relevance  Incidence rates for MCC in Queensland are at least double those of any that have been previously published elsewhere in the world. It is likely that Queensland’s combination of a predominantly white population, outdoor lifestyle, and exposure to sunlight has played a role in this unwanted result. Interventions are required to increase awareness of MCC among clinicians and the public.

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Figure 1.
Incidence Rate Trends for Merkel Cell Carcinoma, Queensland, Australia, 1993-2010

Trends calculated by sex (A), age at diagnosis (B), body site (C), and stage at diagnosis (D) using Joinpoint regression, based on rates that were directly age standardized to the 2000 United States Standard Population.21Head includes the face, ears, scalp, and neck. Other body site includes the trunk, upper limbs, shoulders, and lower limbs. Stage at diagnosis defined according to the criteria set out by the American Joint Committee on Cancer.17

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Figure 2.
Unadjusted Relative Survival Curves for Merkel Cell Carcinoma, Queensland, 2006-2010

Survival by sex (A), age at diagnosis (B), body site (C), and stage at diagnosis (D) calculated using the period method for persons who were at risk of mortality due to Merkel cell carcinoma from January 2006 to December 2010. Stage at diagnosis defined according to the criteria set out by the American Joint Committee on Cancer.17

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