Fam Cancer. 2012 Jun 9. [Epub ahead of print]
Abstract
Lynch
syndrome (LS) is an autosomal dominant cancer syndrome including
increased life-long risk for colorectal (CRC) and endometrial (EC)
cancer, but also for cancers of other types. The risk for CRC is up to
70-80 % and for EC up to 50-60 %. Due to screening and early diagnosing
the mortality related to CRC and EC seems to be low. In spite of many
studies on surveillance of mutation carriers, there is no comprehensive
evaluation on causes of death in LS families. The disease history and
cause of death of all the deceased, tested mutation carriers and their
mutation negative relatives in the Finnish LS families (N = 179) was
examined utilizing hospital records and relevant national registries.
Out of 1069 mutation carriers 151 had succumbed; 97 (64 %) from cancer.
Out of 1146 mutation-negative family 44 members had died; 11 (25 %) of
them from cancer. In 12 (7.7 %) of the deceased mutation carriers no
cancer had been diagnosed. The mean age of death from cancer was
63.2 years vs. 68.8 years from non-cancer causes. Only 7.9 % of the
patients with CRC had died from CRC and 5 % of those with EC,
respectively. 61 % of the cancer deaths were related to extra-colonic,
extra-endometrial cancers. The cumulative overall and cancer specific
death rates were significantly increased in Mut+ compared to Mut- family
members. Even surveillance yields decrease in the life-long risk and
mortality of the most common cancers CRC and EC in LS, almost all
mutation carriers will contract with cancer, and two thirds of the
deceased have died from cancer. This should be taken in account in
genetic counseling. Mutation carriers should be encouraged to seek help
for abnormal symptoms.
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