Are patients with multiple endocrine neoplasia type I prone to premature death?

Multiple endocrine neoplasia type I (MEN-I) is an autosomal dominant disord er characterized by endocrinopathies involving the anterior pituitary gland , parathyroid glands, and pancreas. The long-term prognosis for patients af fected with this disorder is uncertain. To better characterize this prognos is, we performed a retrospective review of all patients with MEN-I treated at a single institution during the period 1951-1997, A group of 233 patient s served as the study population. Their records were analyzed for confirmat ion of diagnosis, treatments received, long-term survival, and cause of dea th. Altogether, 108 eight male patients (46%) and 125 female patients (54%) were identified. At the conclusion of the study, 164 (70%) were alive and 69 (30%) were deceased, with a median follow-up for patients alive at last contact of 13.4 years (range < 1 month to 54.3 years). The cause of death w as reliably obtained in 60 patients. Of these patients, 17 (28%) died of ca uses related to MEN-I, most commonly metastatic islet cell tumors (10 patie nts). The remaining patients died of causes unrelated to MEN-I, most common ly coronary artery disease and nonendocrine malignancies (14% each). The ov erall 20-year survival of MEN-I patients was 64% (95% CI was 56-72%), and t hat of an age- and gender-matched upper Midwest population was 81% (p < 0.0 01). Patients with MEN-I appear to be at increased risk of premature death. Earlier diagnosis and treatment of potentially malignant pancreatic islet cell neoplasms may result in a decrease of this premature mortality.