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

Figure

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  Fig. 1. Clinical manifestation findings. (A) X-ray examination of the kidneys, ureter, and bladder showed calcified stones (arrows) in both ureters.(B, C) Abdominal nonenhanced CT showed calcified stones (arrows) in both ureters.

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  Fig. 2. Pancreatic gastrinoma findings. (A) Abdominal CT showed multiple restricted nodular masses (arrows). (B) Liver MRI showed hypersignal enhanced lesion in the superior segment of the left lobe. (C) EUS showed well defined hypoechoic nodular lesion. (D) Octerotide scan showed focal hyperactivity in the pancreas head and body portion and mild focal hyperactivity in the left hepatic lobe (arrows). (E) PET-CT showed 18F 2-fluoro-2-deoxyglucose (FDG) hot uptake in pancreatic area (arrow).

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  Fig. 3. Multiple endocrine neoplasia type I findings. (A) Thyroid ultrasonography showed parathyroid adenoma in right superior pole of thyroid.(B) Parathyroid Tc-99m methoxy isobutyl isonitrile scan showed persistent tracer in right thyroid lobe (arrow; after 30 minutes, 60 minutes). (C) Brain MRI showed low attenuation of pituitary such as microadenoma (arrow).

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  Fig. 4. Pathologic finding. The gastrinoma cells were positive by immunohistochemocal stain of gastrin (×400).

Reference

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