Multiple endocrine neoplasm, type 1. Gastrinomas, pancreatic neoplasms, microcarcinoids, the Zollinger-Ellison syndrome, lymph nodes, and hepatic metastases
J. J. Shepherd, D. R. Challis, P. F. Davies, J. P. McArdle, B. T. Teh and S. Wilkinson
Department of Surgery, University of Tasmania, Hobart, Australia.
OBJECTIVE: We reviewed the age of presentation, malignant potential, and
outcome of gastrinomas and pancreatic tumors in patients with multiple
endocrine neoplasm, type 1. DESIGN: Screening of one very large and one
smaller, possibly related family on an island, including serum gastrin
estimations and, when indicated, pancreatic ultrasound. SETTING AND
PATIENTS: Over 2000 family members and their family physicians were advised
on screening procedures. INTERVENTION: Data were collected and reviewed
retrospectively and prospectively for all medical records, investigations,
surgical procedures, and available tissue samples. OUTCOME MEASUREMENTS:
Criteria for diagnosis were established for radiological, biochemical, and
histological studies. RESULTS: Sixty-two patients had evidence of
gastrinoma or pancreatic neoplasm. In 19 patients the diagnosis was based
on demonstration of a tumor. In 21 patients the diagnosis was based on
elevated serum gastrin concentration in the absence of demonstrable tumor.
None of these patients required gastric surgery if they first underwent
parathyroidectomy. In 18 patients the diagnosis was based on the
combination of demonstrated pancreatic tumor plus elevated glucagon (two
patients), gastrin (11 patients), or insulin (five patients) concentration.
Peptic ulcer was difficult to control in seven of the 11 patients with
elevated gastrin concentrations plus demonstrated tumor. Four patients had
liver metastases that appeared to be secondary to the pancreatic
gastrinoma. In patients with insulinomas, the first symptoms occurred
before age 20 years. Elevated serum gastrin concentrations were not seen
before age 24 years and were observed to occur for the first time in two
patients after age 50 years.