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Achlorhydria, Parietal Cell Hyperplasia, and Multiple Gastric Carcinoids

A New Disorder

Abraham, Susan C, MD*; Carney, J Aidan, MD*; Ooi, Akishi, MD; Choti, Michael A, MD; Argani, Pedram, MD§

The American Journal of Surgical Pathology: July 2005 - Volume 29 - Issue 7 - p 969-975
doi: 10.1097/01.pas.0000163363.86099.9f
Case Report
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We describe a 54-year-old woman who had multiple gastric carcinoid tumors arising in the setting of marked hypergastrinemia associated with a lack of acid production by hypertrophic parietal cells. The serum gastrin level was 1,400 pg/mL, and investigation revealed no evidence for either of the recognized causes for hypergastrinemia-associated carcinoids, autoimmune gastritis, and Zollinger-Ellision syndrome. Partial gastrectomy was performed. Pathologic examination showed multiple intramucosal and invasive carcinoid tumors of the body and fundus in a background of marked ECL cell hyperplasia. There were no gastric or duodenal ulcerations. One perigastric lymph node was metastatically involved. The oxyntic mucosa showed marked hyperplasia and hypertrophy of the parietal cells. Some of these cells were vacuolated, and many displayed protrusions of apical cytoplasm into dilated oxyntic glands filled with inspissated eosinophilic material. Similar findings have occurred in 1 other patient, strongly indicating that the clinicopathologic alterations in the 2 cases are not random but, on the contrary, represent a very rare disorder of gastric carcinoids associated with an intrinsic acid secretion abnormality of the parietal cells.

From the *Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN; †Department of Pathology, School of Medicine, Kanazawa University, Kanazawa, Japan; and Departments of ‡Surgery and §Pathology, Johns Hopkins University School of Medicine, Baltimore, MD.

Reprints: Susan C. Abraham, MD, Department of Pathology, Hilton 11, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (e-mail: abraham.susan@mayo.edu).

© 2005 Lippincott Williams & Wilkins, Inc.