Does familial non-medullary thyroid cancer adversely affect survival?
BACKGROUND
Familial non-medullary thyroid cancer (FNMTC) is associated with a higher rate of multifocality and a higher recurrence rate than sporadic thyroid cancer. However, the effect of FNMTC on life expectancy is unknown.


METHODS
Using data from our FNMTC database, we calculated life expectancy and survival rates after diagnosis of FNMTC and compared the results with the rates for unaffected family members and for the standard US population. Overall life expectancy and survival rates were calculated using the Kaplan-Meier method. We compared patients from families with 2 affected members with patients from families with > or = 3 affected members. We also compared patients diagnosed in a known familial setting (index cases and subsequent cases) with patients diagnosed before the familial setting was recognized.


RESULTS
There were 139 affected patients with 757 unaffected family members. The mean age at diagnosis was 40.8 +/- 13.9 years and the mean follow-up time was 9.4 +/- 11.7 years. Ten patients died of thyroid cancer during follow-up. The life expectancy of patients with FNMTC was similar to that of their unaffected family members. Survival was significantly shorter for patients with 3 or more affected family members, for patients diagnosed before the familial setting was recognized, and for patients with anaplastic cancer.


CONCLUSIONS
Our results suggest that FNMTC may be more aggressive than sporadic thyroid cancer, particularly in families with 3 or more affected members. However, when recognized and treated appropriately, it does not significantly shorten the overall life expectancy of the affected patients.
Triponez
Frederic
F
Advanced Research Technologies, Applied Biosystems, 850 Lincoln Center Drive, MS 447, Foster City, CA 94404-1105, USA. frederic.triponez@hcuge.ch
Wong
Mariwil
M
Sturgeon
Cord
C
Caron
Nadine
N
Ginzinger
David G
DG
Segal
Mark R
MR
Kebebew
Electron
E
Duh
Quan-Yang
QY
Clark
Orlo H
OH
D018263:Adenocarcinoma, Follicular / Q000401:mortality / Q000473:pathology; D000231:Adenocarcinoma, Papillary / Q000401:mortality / Q000473:pathology; D018249:Adenoma, Oxyphilic / Q000401:mortality / Q000473:pathology; D000328:Adult; D030342:Genetic Diseases, Inborn; D006801:Humans; D008017:Life Expectancy; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D013964:Thyroid Neoplasms / Q000401:mortality* / Q000473:pathology*