Cause of death in multiple endocrine neoplasia type 1.
OBJECTIVE
Little data are available on the natural history of untreated multiple endocrine neoplasia type 1 (MEN-1). These data are essential in deciding treatment that may carry significant morbidity. We determined the causes of death in a large MEN-1 kindred with data available over a period of 130 years. Most cases were unrecognized as MEN-1 at the time of patient's death.


METHODS
Retrospective study of recorded medical data from 1861 to 1991.


METHODS
One hundred fifty-nine deaths occurred, of which 46 were in individuals classified as "highly probable" of having MEN-1.


RESULTS
Of 46 deaths in those classified as "highly probable" of having MEN-1, 20 (43.5%) died of a recognized complication of MEN-1 (12 of malignant neoplasms, six of renal calculi, and two of peptic ulcer). If accidental deaths are excluded, 50% of the deaths in patients with MEN-1 were the result of MEN-1, and the mean age of death (50.9 years)was significantly younger than that of other family members.


CONCLUSIONS
It is concluded that MEN-1 leads to premature death, and that neoplasia rather than peptic ulcer disease is the main cause of death. Deaths from pituitary tumor or malignant endocrine tumors within the thorax were just as common or more common than deaths from pancreatic malignant neoplasms.
Wilkinson
S
S
Department of Surgery, University of Tasmania, Hobart, Australia.
Teh
B T
BT
Davey
K R
KR
McArdle
J P
JP
Young
M
M
Shepherd
J J
JJ
D000328:Adult; D000368:Aged; D002423:Cause of Death; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000235:genetics / Q000401:mortality*; D012189:Retrospective Studies; D012307:Risk Factors; D013648:Tasmania / Q000453:epidemiology