CAUSE OF DEATH IN MULTIPLE ENDOCRINE NEOPLASIA TYPE-1

Objective: Little data are available on the natural history of untreat ed multiple endocrine neoplasia type 1 (MEN-1). These data are essenti al in deciding treatment that may carry significant morbidity. We dete rmined the causes of death in a large MEN-1 kindred with data availabl e over a period of 130 years. Most cases were unrecognized as MEN-1 at the time of patient's death. Design: Retrospective study of recorded medical data from 1861 to 1991. Patients: One hundred fifty-nine death s occurred, of which 46 were in individuals classified as ''highly pro bable'' 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 c omplication of MEN-1 (12 of malignant neoplasms, six of renal calculi, and two of peptic ulcer). If accidental deaths are excluded, 50% of t he deaths in patients with MEN-1 were the result of MEN-1, and the mea n age of death (50.9 years) was significantly younger than that of oth er family members. Conclusions: It is concluded that MEN-1 leads to pr emature death, and that neoplasia rather than peptic ulcer disease is the main cause of death. Deaths from pituitary tumor or malignant endo crine tumors within the thorax were just as common or more common than deaths from pancreatic malignant neoplasms.