abstract
BACKGROUND:
The
lynch syndrome (LS) tumor spectrum involves colorectal cancer (CRC),
endometrial cancer (EC), and less frequently various extracolonic
non-endometrial cancers (non-EC).
The organ-specific survival rates of
these patients are well defined, however, the collective survival of
all-cancers combined (CRC + EC + non-EC) are unclear.
METHODS:
Fifty-two
MSH2 patients and 68 MLH1 patients were followed for a median of 6.3
years after diagnosis of
first cancer, regardless of type. The
proportions of CRC only, EC, non-EC, and multiple primary cancers were
compared between the two genotypes. Kaplan-Meier curves were developed
for survival comparisons.
RESULTS:
MSH2 patients present
less frequently with only CRC (37% MSH2, 62% MLH1, P = 0.0096),
manifest
more multiple primary cancers (38% MSH2, 18% MLH1, P = 0.013),
develop
more extracolonic cancers (62% MSH2, 38% MLH1, P = 0.003), non-EC only
cancers (46% MSH2, 24% MLH1, P = 0.028) and carry a
greater risk for
urinary tract cancer (UTC) (13.4% MSH2, 1.5% MLH1, P = 0.024). There was
no difference in 10-year survival between the two groups (P = 0.4).
CONCLUSION:
The
additional propensity for UTC in MSH2 carriers argues in favor of UTC
screening in MSH2 individuals. Other types of cancer screening should be
tailored to the expression history of the specific LS mutation.
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