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Cook, Sam, Pethick, Joanna, Kibbi, Nour, Hollestein, Loes, Lavelle, Katrina, de Vere Hunt, Isabella, Turnbull, Clare, Rous, Brian, Husain, Akhtar, Burn, John, Lüchtenborg, Margreet, Santaniello, Francesco, McRonald, Fiona, Hardy, Steven, Linos, Eleni, Venables, Zoe 
ORCID: https://orcid.org/0000-0002-9929-2693 and Rajan, Neil
(2023)
Sebaceous carcinoma epidemiology, associated malignancies and Lynch/Muir-Torre syndrome screening in England from 2008 to 2018.
Journal of the American Academy of Dermatology, 89 (6).
pp. 1129-1135.
ISSN 0190-9622
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Abstract
Background: Sebaceous carcinomas (SC) may be associated with the cancer predisposition syndrome Muir-Torre/Lynch syndrome (MTS/LS), identifiable by SC mismatch repair (MMR) screening; however, there is limited data on MMR status of SC. Objective: To describe the epidemiology of SC, copresentation of other cancers, and population level frequency of MMR screening in SC. Methods: A population-based retrospective cohort study of SC patients in the National Cancer Registration and Analysis Service in England. Results: This study included 1077 SC cases (739 extraocular, 338 periocular). Age-standardized incidence rates (ASIR) were higher in men compared with women, 2.74 (95% CI, 2.52-9.69) per 1,000,000 person-years for men versus 1.47 person-years (95% CI, 1.4-1.62) for women. Of the patients, 19% (210/1077) developed at least one MTS/LS-associated malignancy. MMR immunohistochemical screening was performed in only 20% (220/1077) of SC tumors; of these, 32% (70/219) of tumors were MMR deficient. Limitations: Retrospective design. Conclusions: Incorporation of MMR screening into clinical practice guidelines for the management of SC will increase the opportunity for MTS/LS diagnoses, with implications for cancer surveillance, chemoprevention with aspirin, and immunotherapy treatment targeted to MTS/LS cancers.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | dermatopathology,epidemiology,genetics,lynch syndrome,mismatch repair deficiency,muir-torre syndrome,sebaceous carcinoma,dermatology,sdg 3 - good health and well-being ,/dk/atira/pure/subjectarea/asjc/2700/2708 |
| Faculty \ School: | Faculty of Medicine and Health Sciences > Norwich Medical School |
| UEA Research Groups: | Faculty of Medicine and Health Sciences > Research Centres > Population Health (former - to 2025) Faculty of Science > Research Groups > Norwich Epidemiology Centre Faculty of Medicine and Health Sciences > Research Groups > Norwich Epidemiology Centre Faculty of Medicine and Health Sciences > Research Centres > Public Health |
| Related URLs: | |
| Depositing User: | LivePure Connector |
| Date Deposited: | 19 Mar 2025 13:30 |
| Last Modified: | 18 Jun 2026 20:34 |
| URI: | https://ueaeprints.uea.ac.uk/id/eprint/98805 |
| DOI: | 10.1016/j.jaad.2023.03.046 |
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