Lynch syndrome and sextuple primary malignancies

Abstract

Lynch syndrome or hereditary nonpolyposis colorectal cancer is the most common of hereditary colorectal cancer and accounts for 1–3%. Lynch and Chapelle estimated that it accounts 5–8% for all colorectal cancers. It is an autosomal dominant syndrome characterized by predisposition of various cancers (colorectal, stomach, endometrial, ovarian, renal, small bowel, and hepatobiliary tract) at earlier age than in general population and occurs as a result of mutation in DNA mismatch repair genes. This article presents a rare clinical of a 61-year-old female diagnosed with extracolonic Lynch syndrome with six metachronous tumours acquiring in digestive tract during the period from 1993 to 2014 (over 21 years). No other cases of six primary malignancies in patient with Lynch syndrome have been reported in literature. Upon diagnosis of Lynch syndrome, it is important to screen patient for malignancies of different localization as this syndrome predisposes appearance of various cancers at earlier age than in general population.

Keywords:

• Clinical case
• metachronous cancer
• multiple malignancies
• microsatellite instability
• hereditary nonpolyposis colorectal cancer

Acknowledgements

We would like to thank our patients for their comprehension and cooperation. We also acknowledge the medical staff who were involved in treating these patients.

Disclosure statement

No potential conflict of interest was reported by the authors.

Funding

This work was funded by Vilnius University.