Background: Multiple Endocrine Neoplasia Type 1 (MEN 1) is an autosomal dominant disease predisposing to hyperplasia and neoplasia of parathyroid, pancreatic and pituitary tissue. Patients typically present prior to the age of 30 years and are subject to reduced life expectancy.
Aim: To determine predictors of mortality in MEN 1.
Method: Retrospective cohort study of 170 patients with a common MEN1 genotype. Information was extracted from hospital records. Cox proportional hazards analysis was used to assess median life expectancy (MLE).
Results: Cohort MLE was 65.3 years. Compared with female sex, male sex was associated with decreased survival (MLE 70.4 vs 62.7 years, p = 0.03). The presence of adrenal neoplasia diagnosed prior to, compared with after, age 45 years (MLE 51.8 vs 70.8 years, p < 0.01) and liver metastases diagnosed prior to, compared with after, age 45 years (MLE 38.6 vs 68.9 years, p < 0.01) also predicted survival. However, diagnosis of liver metastases beyond the age of 45 years (MLE 68.9 years) was not associated with a statistically significant difference in survival compared with those without liver metastases (MLE 65.6 years, p = 0.66).
The presence of the following disease manifestations diagnosed prior to, compared with after, age 45 years, was not associated with a statistically significant difference in MLE: primary hyperparathyroidism (MLE 68.9 vs 70.8 years, p = 0.15); pancreatic neuroendocrine tumour (MLE 68.9 vs 66.2 years, p = 0.70); gastrinoma (MLE 60.5 vs 70.4 years, p = 0.17); pituitary adenoma (MLE 72.6 vs 70.8 years, p = 0.32).
Conclusion: Among patients with MEN 1, males and those who are diagnosed with either adrenal neoplasia or liver metastases prior to the age of 45 have a poorer prognosis.