Sebaceous carcinoma epidemiology, associated malignancies and Lynch/Muir-Torre syndrome screening in England from 2008 to 2018

Sam Cook, Joanna Pethick, Nour Kibbi, Loes Hollestein, Katrina Lavelle, Isabella de Vere Hunt, Clare Turnbull, Brian Rous, Akhtar Husain, John Burn, Margreet Lüchtenborg, Francesco Santaniello, Fiona McRonald, Steven Hardy, Eleni Linos, Zoe Venables, Neil Rajan*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

3 Citations (Scopus)
27 Downloads (Pure)

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.

Original languageEnglish
Pages (from-to)1129-1135
Number of pages7
JournalJournal of the American Academy of Dermatology
Volume89
Issue number6
Early online date7 Apr 2023
DOIs
Publication statusPublished - Dec 2023

Bibliographical note

Funding Information:
Funding sources: NR’s research is supported by the Newcastle NIHR Biomedical Research Centre (BRC).

Publisher Copyright:
© 2023 American Academy of Dermatology, Inc.

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