Multiple endocrine neoplasm, type 1. Gastrinomas, pancreatic neoplasms, microcarcinoids, the Zollinger-Ellison syndrome, lymph nodes, and hepatic metastases.
OBJECTIVE
We reviewed the age of presentation, malignant potential, and outcome of gastrinomas and pancreatic tumors in patients with multiple endocrine neoplasm, type 1.


METHODS
Screening of one very large and one smaller, possibly related family on an island, including serum gastrin estimations and, when indicated, pancreatic ultrasound.


METHODS
Over 2000 family members and their family physicians were advised on screening procedures.


METHODS
Data were collected and reviewed retrospectively and prospectively for all medical records, investigations, surgical procedures, and available tissue samples.


METHODS
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.
Shepherd
J J
JJ
Department of Surgery, University of Tasmania, Hobart, Australia.
Challis
D R
DR
Davies
P F
PF
McArdle
J P
JP
Teh
B T
BT
Wilkinson
S
S
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D015408:Gastrinoma / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000556:secondary / Q000601:surgery; D005755:Gastrins / Q000097:blood; D005934:Glucagon / Q000097:blood; D006801:Humans; D007328:Insulin / Q000097:blood; D007340:Insulinoma / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000556:secondary / Q000601:surgery; D008113:Liver Neoplasms / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000556:secondary / Q000601:surgery; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000097:blood / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000097:blood / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D011446:Prospective Studies; D012189:Retrospective Studies; D015043:Zollinger-Ellison Syndrome / Q000097:blood / Q000453:epidemiology / Q000473:pathology / Q000601:surgery
D005755:Gastrins; D007328:Insulin; D005934:Glucagon