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abstract
Causes of Cancer Death Among First-Degree Relatives in Japanese Families with Lynch Syndrome
Aim: To elucidate the causes of cancer
death in Japanese families with Lynch syndrome (LS).
Methods: The
distributions of
cancer deaths in 485 individuals from 67 families
with LS (35, 30, and two families with MutL homologue 1 (MLH1), MSH2,
and
MSH6 gene mutations, respectively), obtained from
the Registry of the Japanese Society for Cancer of the Colon and Rectum
were analyzed.
Results: Among 98 cancer deaths of
first-degree relatives of unknown mutation status, 53%, 19%, 13% (among
females), 7% (among females) and 5% were due to
colorectal, gastric, uterine, ovarian, and hepatobiliary cancer,
respectively.
The proportion of deaths from extra-colonic cancer
was significantly higher in families with MSH2 mutation than in those
with
MLH1 mutation (p=0.003).
Conclusion: In addition to
colonic and uterine cancer, management and surveillance targeting
gastric,
ovarian and hepatobiliary cancer are considered
important for Japanese families with LS. Extra-colonic cancer in
families
with MSH2 mutation might require for more intensive
surveillance.
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