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. 2023 Jan;149(1):25-46.
doi: 10.1007/s00432-022-04397-0. Epub 2022 Oct 17.

Risk of cancer in individuals with Lynch-like syndrome and their families: a systematic review

Pandu P Nugroho #  1   2 , Siti Alyaa S Ghozali #  1   2 , Daniel D Buchanan  3   4   5 , Mia I Pisano  6 , Jeanette C Reece  7
Affiliations

Risk of cancer in individuals with Lynch-like syndrome and their families: a systematic review

Pandu P Nugroho et al. J Cancer Res Clin Oncol. 2023 Jan.

Abstract

Background: Lynch-like syndrome (LLS) tumors have similar clinicopathological features to Lynch syndrome (LS) tumors but have no identifiable pathogenic germline mismatch repair gene variant. However, cancer risks in LLS patients and first-degree relatives (FDRs) are not well defined.

Methods: To clarify LLS-associated cancer risks, a systematic review of all studies examining all cancer risks in LLS was performed. Searching of Medline, Embase, Pubmed, Cochrane and CINAHL databases and reference/citation checking identified relevant studies published between January 1, 1980 and February 11, 2021. Joanna Briggs Institute Appraisal Tools assessed the risk of bias.

Results: Six studies (five cohort/one cross-sectional) were eligible for study inclusion. One study found no difference in colorectal cancer (CRC) incidence between LLS and LS patients or CRC risks at aged 70 years. Three studies found CRC incidence in LLS FDRs was higher than the general population but lower than LS FDRs. Two studies showed no difference in CRC diagnosis age between LLS patients and LS patients. Endometrial cancer risks in LLS patients were higher than the general population but lower than LS patients.

Conclusion: Evidence of elevated CRC risks in LLS patients and FDRs supports increased colonoscopy surveillance strategies for LLS patients and FDRs in line with current recommendations for LS. Due to heterogeneity amongst LLS populations, extended intervals between screening may be advised for low-risk families. Studies to resolve the molecular characterization and definition of LLS are needed to clarify cancer risks associated with LLS which in turn may individualize surveillance strategies for LLS patients and families.

Keywords: Colorectal cancer; Extra-colonic cancer; Lynch syndrome; Lynch-like syndrome; Standard incidence ratio.

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Conflict of interest statement

The authors declare no competing interests.

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
PRISMA flow diagram of inclusion of studies
See this image and copyright information in PMC

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