Familial Multiple Endocrine Adenomatosis

Abstract

Six cases of endocrine neoplasia have been found in a kindred with multiple endocrine adenomatosis that has been traced through five generations. One patient had an islet cell adenoma and the Zollinger-Ellison syndrome; a second had islet cell adenomas, the Zollinger-Ellison syndrome, a chromophobe adenoma of the pituitary, adrenal cortical adenomas, probable hyperparathyroidism, and multiple lipomatosis; a third had asymptomatic hyperparathyroidism and thyrotoxicosis; and three patients had asymptomatic hyperparathyroidism alone. The inheritance pattern was that of a dominant autosomal gene with variable expressivity. All patients with hyperparathyroidism had chief cell hyperplasia. The predominance of patients with hyperparathyroidism without other evident endocrine neoplasia in this and similar kindreds indicates that all patients with apparent "familial hyperparathyroidism" should be examined for possible multiple endocrine neoplasia. An interesting feature of the present kindred is that of the 45 members who were traced, three committed suicide.