13 - Multiple Endocrine Adenomatosis; Multiple Hormone-Producing Tumours, a Familial Syndrome

Abstract

A change in nomenclature is suggested for multiple endocrine adenomatosis. In view of the very high incidence of malignancies in the affected organs, the term should be replaced by multiple endocrine neoplasia, type 1 (Wermer’s syndrome). The pathology of the syndrome consists of hyperplasia and tumours of the anterior pituitary, the parathyroid glands, the islets of Langerhans, the adrenal cortex and the thyroid. The changes in the involved glands appear generally in multiplicity. Endocrine symptoms like spontaneous hypoglycaemia, hyperparathyroidism, symptoms of hyper- or hypofunction of the anterior pituitary, Cushing’s and Conn’s syndrome, develop simultaneously or in succession. Peptic ulcers of the Zollinger-Ellison type—the effect of multiple gastrin-producing islet cell tumours—are an integral part of the syndrome. Multiple lipomas are common. Carcinoid tumours, which can originate in various organs, appear frequently and may cause the carcinoid syndrome. In the author’s opinion the syndrome represents the expression of a pleiotropic autosomal gene, activated in the diseased organs and tissues. Cells which have their origin in the neural crest, like parathyroid and islet cells, apparently possess the gene-activating capacity to a high degree, but the adrenal cortex and the adipose tissue, despite different embryological origin, can interact with the tumour-inducing gene equally well. The pathological and clinical entity described by Zollinger and Ellison should be called Zollinger-Ellison disease. It is not inherited and its endocrine pathology is limited to a gastrinoma. For the analogous changes in the gastrointestinal tract in patients with multiple endocrine neoplasia type 1 the designation Zollinger-Ellison syndrome appears appropriate.