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Prophylactic surgery prevents endometrial and ovarian cancer in Lynch syndrome

Nature Clinical Practice Oncology volume 4, pages 672–673 (2007)Cite this article

In 1966, Lynch described a hereditary syndrome of autosomal dominant susceptibility to colorectal cancer (CRC) in relatively young people. This syndrome was initially characterized by proclivity of tumors to the proximal colon, where ∼70% arose to the right of the splenic flexure, in the absence of multiple colonic adenomas but with an excess of synchronous and metachronous primary CRCs.1,2 It soon became evident, however, that there were many extra-colonic cancers integral to the syndrome including cancers of the upper gastrointestinal tract, carcinomas of the small bowel, hepatobiliary tract, pancreas, endometrium, and ovary, transitional cell carcinoma of the upper tract urothelium, brain tumors (predominantly glioblastomas of the Turcot's variant), as well as sebaceous adenomas and carcinomas and multiple keratoacanthomas of the Muir–Torre variant.2 Increased awareness within the medical community that these extra-colonic cancers are integral to this condition has shown the initial term for this syndrome—hereditary nonpolyposis colorectal cancer—to be inadequate and even misleading, and has led to the adoption of the eponymous term 'Lynch syndrome'.

Endometrial carcinoma (EC) has emerged as the second most common cancer associated with Lynch syndrome. Female carriers of a predisposing mutation in a mismatch repair (MMR) gene associated with Lynch syndrome have a lifetime risk for EC of ∼30% to over 60%.1,3,4 Studies have also demonstrated a lifetime risk of 12% for ovarian cancer (OC) in women who carry a deleterious mutation.5,6 In patients with Lynch syndrome over 20% of the cases of OC have been reported to be synchronous with EC.5,7,8 Both EC and OC are now considered to be 'sentinel cancers' for Lynch syndrome, preceding the diagnosis of CRC in about half of the women who develop two or more of these integral tumors.4,6,7 The mean age for the diagnosis of either EC or OC in patients with Lynch syndrome is in the fifth to early sixth decade of life,4,6,7,8 with both cancers reported in some patients as early as the third decade.6,7,8

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References

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Authors and Affiliations

  1. HT Lynch is Professor of Medicine and Chair of the Department of Preventive Medicine and Public Health and MJ Casey is Professor of Obstetrics and Gynecology and Director of Gynecologic Oncology at Creighton University School of Medicine, Omaha, NE, USA.,

    Henry T Lynch & Murray Joseph Casey

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  1. Henry T Lynch
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  2. Murray Joseph Casey
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Correspondence to Henry T Lynch.

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Lynch, H., Casey, M. Prophylactic surgery prevents endometrial and ovarian cancer in Lynch syndrome. Nat Rev Clin Oncol 4, 672–673 (2007). https://doi.org/10.1038/ncponc1002

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