Editorial: The Mystery of Mismatch Repair Deficiency: Lynch or Lynch-like?

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"....The work presented by Rodriguez–Soler and the EPICOLON
group raises the novel concept that there are CRCs
with MSI that are not Lynch syndrome, and not caused by
the acquired hypermethylation of the MLH1 gene. There are
numerous reasons why this may not be the case, principally
based on the difficult challenge of finding all of the ways a
gene can undergo inactivation. However, the different clinical
features in the family members of those with LLS suggest
that there may be some other mechanism for generating
DNA MMR deficiency and MSI. Perhaps LLS group is heterogeneous
and contains some true Lynch syndrome—and
something else. Time will tell."