Sebaceous carcinoma epidemiology, associated malignancies and Lynch/Muir-Torre syndrome screening in England from 2008 to 2018.
J Am Acad Dermatol
; 89(6): 1129-1135, 2023 12.
Article
em En
| MEDLINE
| ID: mdl-37031776
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.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Neoplasias das Glândulas Sebáceas
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Carcinoma Basocelular
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Neoplasias Colorretais
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Neoplasias de Anexos e de Apêndices Cutâneos
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Adenocarcinoma Sebáceo
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Síndrome de Muir-Torre
Tipo de estudo:
Diagnostic_studies
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Guideline
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Observational_studies
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Risk_factors_studies
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Screening_studies
Limite:
Female
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Humans
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Male
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article