A case of Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1 with urolithiasis as the initial presentation

• Published in: The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi Vol. 61; no. 6; p. 333
• Main Authors: Lee, Na Eun, Lee, Young Jae, Yun, So Hee, Lee, Jae Un, Park, Moon Sik, Kim, Joong Keun, Kim, Ji Woong, Cho, Jin Woong
• Format: Journal Article
• Language: English
• Published: Korea (South) 01.06.2013
• Subjects: Gastrinoma - diagnostic imaging; Gastrinoma - metabolism; Gastrinoma - pathology; Gastrins - metabolism; Humans; Immunohistochemistry; Liver - diagnostic imaging; Magnetic Resonance Imaging; Male; Mesenteric Artery, Superior - diagnostic imaging; Middle Aged; Multimodal Imaging; Multiple Endocrine Neoplasia Type 1 - complications; Multiple Endocrine Neoplasia Type 1 - diagnosis; Multiple Endocrine Neoplasia Type 1 - diagnostic imaging; Pancreas - diagnostic imaging; Pituitary Gland - diagnostic imaging; Positron-Emission Tomography; Radiopharmaceuticals; Thyroid Gland - diagnostic imaging; Tomography, X-Ray Computed; Ultrasonography; Urolithiasis - diagnosis; Urolithiasis - etiology; Zollinger-Ellison Syndrome - complications; Zollinger-Ellison Syndrome - diagnosis

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.