Survival of MUTYH-associated polyposis patients with colorectal cancer and matched control colorectal cancer patients.
BACKGROUND
MUTYH-associated polyposis is a recessively inherited disorder characterized by a lifetime risk of colorectal cancer that is up to 100%. Because specific histological and molecular genetic features of MUTYH-associated polyposis colorectal cancers might influence tumor behavior and patient survival, we compared survival between patients with MUTYH-associated polyposis colorectal cancer and matched control patients with colorectal cancer from the general population.


METHODS
In this retrospective multicenter cohort study from Europe, 147 patients with MUTYH-associated polyposis colorectal cancer were compared with 272 population-based control patients with colorectal cancer who were matched for country, age at diagnosis, year of diagnosis, stage, and subsite of colorectal cancer. Kaplan-Meier survival and Cox regression analyses were used to compare survival between patients with MUTYH-associated polyposis colorectal cancer and control patients with colorectal cancer. All statistical tests were two-sided.


RESULTS
Five-year survival for patients with MUTYH-associated polyposis colorectal cancer was 78% (95% confidence interval [CI] = 70% to 84%) and for control patients was 63% (95% CI = 56% to 69%) (log-rank test, P = .002). After adjustment for differences in age, stage, sex, subsite, country, and year of diagnosis, survival remained better for MUTYH-associated polyposis colorectal cancer patients than for control patients (hazard ratio of death = 0.48, 95% CI = 0.32 to 0.72).


CONCLUSIONS
In a European study cohort, we found statistically significantly better survival for patients with MUTYH-associated polyposis colorectal cancer than for matched control patients with colorectal cancer.
Department of Clinical Genetics, Leiden University Medical Center, the Netherlands. m.nielsen@lumc.nl
Maartje
M
Nielsen
Liza N
LN
van Steenbergen
Natalie
N
Jones
Stefanie
S
Vogt
Hans F A
HF
Vasen
Hans
H
Morreau
Stefan
S
Aretz
Julian R
JR
Sampson
Olaf M
OM
Dekkers
Maryska L G
ML
Janssen-Heijnen
Frederik J
FJ
Hes
D000328:Adult; D000368:Aged; D015982:Bias; D016022:Case-Control Studies; D003111:Colonic Polyps / Q000150:complications / Q000235:genetics* / Q000276:immunology / Q000401:mortality*; D015179:Colorectal Neoplasms / Q000235:genetics* / Q000276:immunology / Q000401:mortality*; D045647:DNA Glycosylases / Q000235:genetics*; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D015983:Selection Bias
D045647:DNA Glycosylases; C061738:mutY adenine glycosylase