4.2 Review

Prospective observational data informs understanding and future management of Lynch syndrome: insights from the Prospective Lynch Syndrome Database (PLSD)

Journal

FAMILIAL CANCER
Volume 20, Issue 1, Pages 35-39

Publisher

SPRINGER
DOI: 10.1007/s10689-020-00193-2

Keywords

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

Funding

  1. University of Helsinki including Helsinki University Central Hospital
  2. Emil Aaltonen Foundation
  3. Finnish Medical Foundation
  4. Sigrid Juselius Foundation

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Research suggests that current guidelines for managing Lynch syndrome should be revised based on the latest reports from the PLSD, taking into account the different gene and gender-specific cancer risks and the favorable prognosis associated with the most common cancers.
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.

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