A possible pre-zollinger-ellison state: report on a novel lesion.

OBJECTIVE

To describe a patient with a potential harbinger of Zollinger-Ellison syndrome.

METHODS

We present a case of a 29-year-old woman with persistent galactorrhea and gastric distress, who was a member of an extensive multiple endocrine neoplasia (MEN) I kindred. Both clinical and laboratory findings are reviewed.

RESULTS

Laboratory studies were notable for persistent hypercalcemia and increased levels of parathyroid hormone and fasting pancreatic polypeptide. Because of nonspecific upper gastrointestinal distress, which was sometimes responsive to antacids, the patient underwent further diagnostic evaluation. Baseline gastrin levels were normal. A secretin challenge test yielded normal results. At upper endoscopy, visual findings in the stomach and duodenum were unremarkable. A raised nodule (4 by 2 by 2 mm) was removed from an otherwise normal-appearing duodenum as a routine biopsy procedure. The acinar architecture, the inconspicuous nucleoli, and the granular, eosinophilic cytoplasm of the cells were diagnostic of a neuroendocrine tumor. Histologic immunohistochemical staining was positive for gastrin but negative for pancreatic polypeptide. RNA studies were not technically possible. Subsequently, serum pancreatic polypeptide levels normalized. The patient ultimately had hyperplastic parathyroid glands resected.

CONCLUSION

The duodenal lesion may be a very early gastrinoma, a preclinical manifestation of the Zollinger-Ellison syndrome. The role of early resection of such lesions remains to be determined.