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The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

Division of Endocrinology and Metabolism, University of Florida, College of Medicine Gainesville, Florida 32610

Correspondence: Address requests for reprints to: Dr. Peter W. Stacpoole, Division of Endocrinology and Metabolism, University of Florida, College of Medicine, Gainesville, Florida 32610.

Abstract

Islet cell tumors of the pancreas have been described for over 50 yr. Although carcinoma arising from islet tissue had been noted since the turn of the century (1), not until 1927 was the first case of a hormonally active pancreatic neoplasm, an insulinoma, recorded (2). For many years thereafter, pancreatic endocrine tumors were characterized as either insulin-producing or "nonfunctional," the latter being derived from cells apparently devoid of clinically significant hormonal activity. With time, however, other islet cell secretory products were discovered and their tumors identified with various clinical syndromes. Whereas the hypoglycemia of insulinomas and hyperacidity of gastrinomas figured prominently in the initial recognition of these neoplasms, the clinical signs of glucagon-producing tumors were less readily apparent and not as easily attributable to specific hormone excess.

Becker and co-workers (3) are credited with the first description of a glucagonoma. Their case, reported in 1947 but diagnosable only in retrospect, typifies what is now regarded as a classic clinical presentation of the syndrome.

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