title: Are patients with multiple endocrine neoplasia type I prone to premature death?
abstract: Multiple endocrine neoplasia type I (MEN-I) is an autosomal dominant disorder characterized by endocrinopathies involving the anterior pituitary gland, parathyroid glands, and pancreas. The long-term prognosis for patients affected with this disorder is uncertain. To better characterize this prognosis, 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 patients served as the study population. Their records were analyzed for confirmation of diagnosis, treatments received, long-term survival, and cause of death. 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 was reliably obtained in 60 patients. Of these patients, 17 (28%) died of causes related to MEN-I, most commonly metastatic islet cell tumors (10 patients). The remaining patients died of causes unrelated to MEN-I, most commonly coronary artery disease and nonendocrine malignancies (14% each). The overall 20-year survival of MEN-I patients was 64% (95% CI was 56-72%), and that of an age- and gender-matched upper Midwest population was 81% (p < 0.001). 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.
authors.affiliation: Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA.
authors.forename: P G
authors.initials: PG
authors.lastname: Dean
authors.forename: J A
authors.initials: JA
authors.lastname: van Heerden
authors.forename: D R
authors.initials: DR
authors.lastname: Farley
authors.forename: G B
authors.initials: GB
authors.lastname: Thompson
authors.forename: C S
authors.initials: CS
authors.lastname: Grant
authors.forename: W S
authors.initials: WS
authors.lastname: Harmsen
authors.forename: D M
authors.initials: DM
authors.lastname: Ilstrup
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D002423:Cause of Death; D002648:Child; D016001:Confidence Intervals; D005260:Female; D006801:Humans; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D016019:Survival Analysis; D013997:Time Factors; D014481:United States / Q000453:epidemiology