Sebaceous carcinoma epidemiology, associated malignancies and Lynch/Muir-Torre syndrome screening in England from 2008 to 2018
Sam Cook, Joanna Pethick, Nour Kibbi, Loes M. Hollestein, Katrina Lavelle, Isabella de Vere Hunt, Clare Turnbull, Brian Rous, Akhtar Husain, John Burn, Margreet Lüchtenborg, Francesco Santaniello, Fiona E. McRonald, Steven J. Hardy, Eleni Linos, Zoe C Venables, Neil Rajan
Abstract
BackgroundSebaceous 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.ObjectiveTo describe the epidemiology of SC, copresentation of other cancers, and population level frequency of MMR screening in SC.MethodsA population-based retrospective cohort study of SC patients in the National Cancer Registration and Analysis Service in England.ResultsThis 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.LimitationsRetrospective design.ConclusionsIncorporation 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. 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. To describe the epidemiology of SC, copresentation of other cancers, and population level frequency of MMR screening in SC. A population-based retrospective cohort study of SC patients in the National Cancer Registration and Analysis Service in England. 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. Retrospective design. 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.