The UMD-MEN1 mutations database
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Multiple endocrine neoplasia syndrome 1 (MEN1) is an autosomal dominant disorder that predisposes carriers to endocrine tumors. The prevalence has been estimated between 1 out of 30 000 and 1 out 100 000. The tumors are mainly developed from endocrine tissues and may arise from parathyroid glands (90-100%), pancreas (50-70%), pituitary gland (20-40%), adrenal glands (20-40%) and at lower frequency from the bronchi and thymus (<10%) (1). The heterogeneity of tumor penetrance was marked, ranging from 9 to 25 years for the earliest, and from 68 years to 77 years for the latest tumor manifestation (2).

Pituitary disease is associated with hyperparathyroidism in 90% of cases, with enteropancreatic tumors in 47% with adrenal tumors in 16% and with thoracic neuroendocrine tumors in 4% (1).

Distribution of the main endocrine lesions (hyperparathyroidism, endocrine enteropancreatic tumors , and pituitary disease) among the 324MEN1 patients. For each endocrine lesion, the number of cases and the percentage of the affected subjects among the total MEN1 patients are given [308 patients with hyperparayhyroidism, 174 with endocrine enteropancreatic tumors,136 with pituitary adenomas, 54 with adrenal tumors, and 25 with neuroendocrine tumors(thymic and bronchial)]. Adrenal and neuroendocrine tumors are not in the figure. Among the 54 adrenal tumors, 8 are associated with parathyroid adenoma (PTH) alone , 21 are associated with both PTH + enteropancreatic tumor (PAN), 2 with both PTH+ Pituitary adenoma (PIT), 2 with both PAN+PIT, 16 with PTH+PAN+PIT, 3 with PTH+PAN+ neuroendocrine tumors (NEUR) and 2 with PTH+PAN+PIT+NEUR. Among the 25 neuroendocrine tumors, 2 are isolated, 2 are associated with pth alone, 11 are associated with both PTH+PAN,1 with both PTH+adrenal tumor (ADR), 3 with PTH+PAN+PIT. 3 WITH PTH+PAN+ADR and 2 with PTH+PAN+PIT+ADR.