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ToolsCook, 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 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 Faculty of Science > Research Groups > Norwich Epidemiology Centre Faculty of Medicine and Health Sciences > Research Groups > Norwich Epidemiology Centre |
| Related URLs: | |
| Depositing User: | LivePure Connector |
| Date Deposited: | 19 Mar 2025 13:30 |
| Last Modified: | 28 Mar 2025 13:15 |
| URI: | https://ueaeprints.uea.ac.uk/id/eprint/98805 |
| DOI: | 10.1016/j.jaad.2023.03.046 |
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