Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome.
In patients with multiple endocrine neoplasia type 1 (MEN-1), gastrinomas are common and thought to occur predominantly in the pancreas. We describe eight patients with MEN-1 and hypergastrinemia (seven with the Zollinger-Ellison syndrome) in whom we searched for neuroendocrine tumors in the pancreas and duodenum. Tumors were found in the proximal duodenum in all eight patients: solitary tumors (diameter, 6 to 20 mm) in three patients and multiple microtumors (diameter, 2 to 6 mm) in the other five. Paraduodenal lymph-node metastases were detected in four patients. Immunocytochemical analysis revealed the presence of gastrin in all the duodenal tumors and in their lymph-node metastases. In contrast, no immunoreactivity for gastrin was present in the endocrine tumors found in the seven pancreatic specimens available for study, except for one tumor with scattered gastrin-positive cells. In four of the six patients whose duodenal gastrinomas were removed, serum gastrin levels returned to normal; in the other two patients gastrin concentrations decreased toward normal. We conclude that in patients with MEN-1 and the Zollinger-Ellison syndrome, gastrinomas occur in the duodenum, but the tumors may be so small that they escape detection.
Pipeleers-Marichal
M
M
Department of Pathology, Academic Hospital, Vrije Universiteit Brussel, Belgium.
Somers
G
G
Willems
G
G
Foulis
A
A
Imrie
C
C
Bishop
A E
AE
Polak
J M
JM
Häcki
W H
WH
Stamm
B
B
Heitz
P U
PU
D000328:Adult; D000368:Aged; D004379:Duodenal Neoplasms / Q000473:pathology*; D005260:Female; D015408:Gastrinoma / Q000473:pathology*; D005755:Gastrins / Q000097:blood; D006801:Humans; D007150:Immunohistochemistry; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000473:pathology; D015043:Zollinger-Ellison Syndrome / Q000473:pathology*
D005755:Gastrins