Are Patients with Multiple Endocrine Neoplasia Type I Prone to Premature Death?

• Published in: World journal of surgery 2000-11, Vol.24 (11), p.1437-1441
• Main Authors: Dean, Patrick G., van Heerden, Jon A., Farley, David R., Thompson, Geoffrey B., Grant, Clive S., Harmsen, W. Scott, Ilstrup, Duane M.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age Distribution; Aged; Biological and medical sciences; Cause of Death; Child; Confidence Intervals; Endocrinopathies; Female; General aspects. Associated endocrine diseases. Endocrine paraneoplasic syndromes; Humans; Life Expectancy; Male; Medical sciences; Middle Aged; Multiple Endocrine Neoplasia Type 1 - diagnosis; Multiple Endocrine Neoplasia Type 1 - mortality; Multiple Endocrine Neoplasia Type 1 - therapy; Retrospective Studies; Risk Assessment; Sex Distribution; Survival Analysis; Time Factors; United States - epidemiology
• ISSN: 0364-2313; 1432-2323
• DOI: 10.1007/s002680010237

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.