A Case of Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 with Urolithiasis as the Initial Presentation

Abstract

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma should be investigated carefully when patients exhibit symptoms associated with hormonal changes. Ureteral stones associated with hyperparathyroidism in the early course of MEN 1 are known to be its most common clinical manifestation; appropriate evaluation and close follow-up of patients with hypercalcemic urolithiasis can lead to an early diagnosis of gastrinoma. We report a patient with ZES associated with MEN 1, and urolithiasis as the presenting entity. A 51-year-old man visited the emergency department with recurrent epigastric pain. He had a history of calcium urinary stone 3 years ago, and 2 years later he had 2 operations for multiple jejunal ulcer perforations; these surgeries were 9 months apart. He was taking intermittent courses of antiulcer medication. Multiple peripancreatic nodular masses, a hepatic metastasis, parathyroid hyperplasia, and a pituitary microadenoma were confirmed by multimodal imaging studies. We diagnosed ZES with MEN 1 and performed sequential surgical excision of the gastrinomas and the parathyroid adenoma. The patient received octreotide injection therapy and close follow-up. (Korean J Gastroenterol 2013;61:333-337)

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

“…In 19 of these cases (95%)[81–94, 96–100], no assessment of gastric acidity was performed. Moreover, there was no standard definition of the diagnosis used and the basis of the diagnosis varied in the different cases.…”

“…Using tumor imaging studies in combination with the hypergastrinemia to diagnose ZES, as is increasingly being done in recently reported cases [82, 84, 87, 88, 90, 94, 110, 134] with patients taking or not taking PPIs, has other limitations. In the presence of PPI therapy, one cannot exclude the presence of appropriate hypergastrinemia if a tumor is seen on cross-sectional imaging studies (CT, ultrasound, or MRI), whether the patient has MEN1 syndrome or not and the mass itself may not even be a NET.…”