Prevalence of Lynch syndrome in women with mismatch repair-deficient ovarian cancer.


Journal

Cancer medicine
ISSN: 2045-7634
Titre abrégé: Cancer Med
Pays: United States
ID NLM: 101595310

Informations de publication

Date de publication:
02 2021
Historique:
received: 14 09 2020
revised: 13 11 2020
accepted: 06 12 2020
pubmed: 29 12 2020
medline: 20 7 2021
entrez: 28 12 2020
Statut: ppublish

Résumé

There are limited data on the prevalence of Lynch syndrome (LS) in women with primary ovarian cancer with mismatch repair deficiency (MMR-D) by immunohistochemistry (IHC). Three hundred and eight cases of primary ovarian, fallopian, and peritoneal cancer between January 2012 and December 2019 were evaluated for MMR-D by IHC. The incidence of LS in this cohort was evaluated. MMR-D by IHC was identified in 16 of 308 (5.2%) (95% CI: 3.2%-8.3%) primary ovarian-related cancers. Most cases with MMR-D were endometrioid (n = 11, 68.7%); (95% CI: 44.2%-86.1%). MSH2/MSH6 protein loss was detected in eight cases (50.0%); (95% CI: 28.0%-72.0%) and MLH1/PMS2 protein loss was detected in four cases (25.0%); (95% CI: 9.7%-50.0%). MSH6 protein loss was detected in two cases (12.5%); (95% CI: 2.2%-37.3%) and PMS2 protein loss was detected in two cases (12.5%); (95% CI: 2.2%-37.3%). All four cases with MLH1/PMS2 protein loss had MLH1 promotor hypermethylation. All 12 women with ovarian cancer suggestive of LS underwent germline testing and 8 (66.6%); (95% CI: 38.8%-86.5%) were confirmed to have LS. Most ovarian cancers with somatic MMR-D were confirmed to have LS in this cohort. Germline testing for LS in addition to BRCA1/2 for all women with an epithelial ovarian cancer would be efficient and would approach 100% sensitivity for identifying Lynch syndrome. Utilization of a multigene panel should also be considered, given the additional non-Lynch germline mutation identified in this cohort.

Sections du résumé

BACKGROUND
There are limited data on the prevalence of Lynch syndrome (LS) in women with primary ovarian cancer with mismatch repair deficiency (MMR-D) by immunohistochemistry (IHC).
MATERIALS AND METHODS
Three hundred and eight cases of primary ovarian, fallopian, and peritoneal cancer between January 2012 and December 2019 were evaluated for MMR-D by IHC. The incidence of LS in this cohort was evaluated.
RESULTS
MMR-D by IHC was identified in 16 of 308 (5.2%) (95% CI: 3.2%-8.3%) primary ovarian-related cancers. Most cases with MMR-D were endometrioid (n = 11, 68.7%); (95% CI: 44.2%-86.1%). MSH2/MSH6 protein loss was detected in eight cases (50.0%); (95% CI: 28.0%-72.0%) and MLH1/PMS2 protein loss was detected in four cases (25.0%); (95% CI: 9.7%-50.0%). MSH6 protein loss was detected in two cases (12.5%); (95% CI: 2.2%-37.3%) and PMS2 protein loss was detected in two cases (12.5%); (95% CI: 2.2%-37.3%). All four cases with MLH1/PMS2 protein loss had MLH1 promotor hypermethylation. All 12 women with ovarian cancer suggestive of LS underwent germline testing and 8 (66.6%); (95% CI: 38.8%-86.5%) were confirmed to have LS.
CONCLUSIONS
Most ovarian cancers with somatic MMR-D were confirmed to have LS in this cohort. Germline testing for LS in addition to BRCA1/2 for all women with an epithelial ovarian cancer would be efficient and would approach 100% sensitivity for identifying Lynch syndrome. Utilization of a multigene panel should also be considered, given the additional non-Lynch germline mutation identified in this cohort.

Identifiants

pubmed: 33369189
doi: 10.1002/cam4.3688
pmc: PMC7897945
doi:

Substances chimiques

Biomarkers, Tumor 0
DNA Repair Enzymes EC 6.5.1.-

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1012-1017

Informations de copyright

© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.

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Auteurs

Rachel Hodan (R)

Cancer Genetics and Genomics, Stanford Health Care, Stanford, CA, USA.
Department of Pediatrics (Genetics), Stanford University School of Medicine, Stanford, CA, USA.

Kerry Kingham (K)

Cancer Genetics and Genomics, Stanford Health Care, Stanford, CA, USA.
Department of Pediatrics (Genetics), Stanford University School of Medicine, Stanford, CA, USA.

Kristina Cotter (K)

Department of Pediatrics (Genetics), Stanford University School of Medicine, Stanford, CA, USA.

Ann K Folkins (AK)

Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.

Allison W Kurian (AW)

Cancer Genetics and Genomics, Stanford Health Care, Stanford, CA, USA.
Department of Oncology, Stanford University School of Medicine, Stanford, CA, USA.
Department of Epidemiology and Population Health, Stanford University School of Medicine, Stanford, CA, USA.

James M Ford (JM)

Cancer Genetics and Genomics, Stanford Health Care, Stanford, CA, USA.
Department of Oncology, Stanford University School of Medicine, Stanford, CA, USA.
Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA.

Teri Longacre (T)

Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.

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