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Cook S, Pethick J, Kibbi N, Hollestein L, Lavelle K, de Vere Hunt I, Turnbull C, Rous B, Husain A, Burn J, Lüchtenborg M, Santaniello F, McRonald F, Hardy S, Linos E, Venables Z, Rajan N. Sebaceous carcinoma epidemiology, associated malignancies and Lynch/Muir-Torre syndrome screening in England from 2008 to 2018. J Am Acad Dermatol. 2023 Apr 7:S0190-9622(23)00536-4. doi: 10.1016/j.jaad.2023.03.046. Epub ahead of print. PMID: 37031776.
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.