Korean J Gastroenterol.  2002 Jan;39(1):50-54.

A Case of Metachronous Multiple Endocrine Neoplasia Type I Manifested as Zollinger-Ellison Syndrome

• Lee YM
• Jung HY
• Choi SY
• Kang HH
• Myung SJ
• Yang SK
• Hong WS
• Kim JH
• Min YI

Abstract

Zollinger-Ellison syndrome (ZES) is characterized by recurrent peptic ulcer and diarrhea caused by gastric acid hypersecretion due to gastrinoma. Approximately, one third of patients with gastrinoma have multiple endocrine neoplasia type I (MEN-I). The diagnosis of gastrinoma is difficult, although it can be cured by surgery after localization. Recently, the somatostatin receptor scintigraphy (SRS) was reported to be more sensitive than any other tumor-localization methods. We report a case of ZES with metachronous MEN-I. A 47-year-old woman had peptic ulcer disease with its complications and recently developed diarrhea. Her serum gastrin level was markedly elevated (1,098 pg/mL) but the results of conventional imaging tests were negative. We confirmed the presence of gastrinoma by the selective intraarterial secretin injection and portal vein sampling. Then, the tumor was eventually located by SRS under the diagnosis of ZES. She underwent a Whipple's operation with the compatible pathologic findings of gastrinoma. She was confirmed to have parathyroid hyperplasia and MEN-I during follow-up.

Keyword

Zollinger-Ellison syndrome; Gastrinoma; Multiple endocrine neoplasia type I (MEN-I); Somatostatin receptor scintigraphy (SRS)

MeSH Terms

Diagnosis
Diarrhea
Female
Follow-Up Studies
Gastric Acid
Gastrinoma
Gastrins
Humans
Hyperplasia
Middle Aged
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Peptic Ulcer
Portal Vein
Radionuclide Imaging
Receptors, Somatostatin
Secretin
Zollinger-Ellison Syndrome*
Gastrins
Receptors, Somatostatin
Secretin