Prospective Observational Data Informs Understanding and Future Management of Lynch Syndrome: Insights From the Prospective Lynch Syndrome Database (PLSD)

Toni T Seppälä 1 2, Mev Dominguez-Valentin 3, Julian R Sampson 4, Pål Møller 3

Affiliations

PMID: 32507935
DOI: 10.1007/s10689-020-00193-2

Abstract

The Prospective Lynch Syndrome Database (PLSD) has been developed as an international, multicentre, prospective, observational study that aims to provide age and organ-specific cancer risks according to gene and gender, estimates of survival after cancer and information on the effects of interventions. Recent reports from PLSD provided improved estimates of cancer risks and survival and showed that different time intervals between surveillance colonoscopies did not affect the incidence, stage or prognosis of colorectal cancer. The PLSD reports suggest that current management guidelines for Lynch syndrome should be revised in light of the different gene and gender-specific cancer risks and the good prognosis for the most commonly associated cancers.In this review, we describe the discrepancies between the current management guidelines for Lynch Syndrome and the most recent prospective observational studies, indicating the areas of further research.

Keywords: Cancer incidence; Colonoscopy; Colorectal cancer; Hereditary non-polyposis colorectal cancer; Lynch syndrome; PLSD; Precision medicine; Prevention; Surveillance.

Similar articles

The Prospective Lynch Syndrome Database reports enable evidence-based personal precision health care.
Møller P.Hered Cancer Clin Pract. 2020 Mar 14;18:6. doi: 10.1186/s13053-020-0138-0. eCollection 2020.PMID: 32190163 Free PMC article. Review.
Lack of association between screening interval and cancer stage in Lynch syndrome may be accounted for by over-diagnosis; a prospective Lynch syndrome database report.
Seppälä TT, Ahadova A, Dominguez-Valentin M, Macrae F, Evans DG, Therkildsen C, Sampson J, Scott R, Burn J, Möslein G, Bernstein I, Holinski-Feder E, Pylvänäinen K, Renkonen-Sinisalo L, Lepistö A, Lautrup CK, Lindblom A, Plazzer JP, Winship I, Tjandra D, Katz LH, Aretz S, Hüneburg R, Holzapfel S, Heinimann K, Valle AD, Neffa F, Gluck N, de Vos Tot Nederveen Cappel WH, Vasen H, Morak M, Steinke-Lange V, Engel C, Rahner N, Schmiegel W, Vangala D, Thomas H, Green K, Lalloo F, Crosbie EJ, Hill J, Capella G, Pineda M, Navarro M, Blanco I, Ten Broeke S, Nielsen M, Ljungmann K, Nakken S, Lindor N, Frayling I, Hovig E, Sunde L, Kloor M, Mecklin JP, Kalager M, Møller P.Hered Cancer Clin Pract. 2019 Feb 28;17:8. doi: 10.1186/s13053-019-0106-8. eCollection 2019.PMID: 30858900 Free PMC article.
Cancer incidence and survival in Lynch syndrome patients receiving colonoscopic and gynaecological surveillance: first report from the prospective Lynch syndrome database.
Møller P, Seppälä T, Bernstein I, Holinski-Feder E, Sala P, Evans DG, Lindblom A, Macrae F, Blanco I, Sijmons R, Jeffries J, Vasen H, Burn J, Nakken S, Hovig E, Rødland EA, Tharmaratnam K, de Vos Tot Nederveen Cappel WH, Hill J, Wijnen J, Green K, Lalloo F, Sunde L, Mints M, Bertario L, Pineda M, Navarro M, Morak M, Renkonen-Sinisalo L, Frayling IM, Plazzer JP, Pylvanainen K, Sampson JR, Capella G, Mecklin JP, Möslein G; Mallorca Group (http://mallorca-group.eu).Gut. 2017 Mar;66(3):464-472. doi: 10.1136/gutjnl-2015-309675. Epub 2015 Dec 9.PMID: 26657901 Free PMC article. Clinical Trial.
Prospective results of surveillance colonoscopy in dominant familial colorectal cancer with and without Lynch syndrome.
Dove-Edwin I, de Jong AE, Adams J, Mesher D, Lipton L, Sasieni P, Vasen HF, Thomas HJ.Gastroenterology. 2006 Jun;130(7):1995-2000. doi: 10.1053/j.gastro.2006.03.018.PMID: 16762622
Recommendations for the care of individuals with an inherited predisposition to Lynch syndrome: a systematic review.
Lindor NM, Petersen GM, Hadley DW, Kinney AY, Miesfeldt S, Lu KH, Lynch P, Burke W, Press N.JAMA. 2006 Sep 27;296(12):1507-17. doi: 10.1001/jama.296.12.1507.PMID: 17003399 Review.