Korean J Gastroenterol.  2013 Jun;61(6):333-337. 10.4166/kjg.2013.61.6.333.

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

• Lee NE
• Lee YJ
• Yun SH
• Lee JU
• Park MS
• Kim JK
• Kim JW
• Cho JW

• KMID: 1718065
• DOI: http://doi.org/10.4166/kjg.2013.61.6.333

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.

Keyword

Zollinger-Ellison syndrome; Multiple endocrine neoplasia type 1; Gastrinoma; Urolithiasis

MeSH Terms

Gastrinoma/metabolism/pathology/ultrasonography
Gastrins/metabolism
Humans
Immunohistochemistry
Liver/radiography
Magnetic Resonance Imaging
Male
Mesenteric Artery, Superior/radiography
Middle Aged
Multimodal Imaging
Multiple Endocrine Neoplasia Type 1/complications/*diagnosis/radiography
Pancreas/radiography/radionuclide imaging
Pituitary Gland/radiography
Positron-Emission Tomography
Radiopharmaceuticals/diagnostic use
Thyroid Gland/ultrasonography
Tomography, X-Ray Computed
Urolithiasis/*diagnosis/etiology
Zollinger-Ellison Syndrome/complications/*diagnosis
Gastrins
Radiopharmaceuticals