The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

A case of Zollinger-Ellison syndrome with MEN-1

식도 협착과 다발성 내분비종 1형을 동반한 졸링거-엘리슨 증후군 1예

  • Kong, Yun-Ho (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Kim, Young-Don (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Han, Koon-Hee (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Lee, Se-Hyung (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Jung, Woo-Jin (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Jang, Hyuk-Jai (Department of General Surgery, University of Ulsan College of Medicine, Gangneung Asan Hospital) ;
  • Cheon, Gab-Jin (Department of Internal Medicine, University of Ulsan College of Medicine, Gangneung Asan Hospital)
  • 공윤호 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 김영돈 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 한군희 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 이세형 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 정우진 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 장혁재 (울산대학교 의과대학 강릉아산병원 일반외과학교실) ;
  • 천갑진 (울산대학교 의과대학 강릉아산병원 내과학교실)
  • Received : 2009.07.29
  • Accepted : 2009.09.25
  • Published : 2010.09.01
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Abstract

Zollinger-Ellison syndrome (ZES) is a clinical syndrome caused by excessive gastric acid secretion by gastrinoma, characteristically causing peptic disease and/or gastroesophageal reflux disease. Approximately one third of patients with gastrinoma have multiple endocrine neoplasia type 1 (MEN-1). A 56-year-old man was admitted for abdominal pain and diarrhea lasting for 2 weeks. The endoscopic findings revealed severe reflux esophagitis and multiple ulcers at the bulb and second portion of the duodenum. He was diagnosed as ZES based on typical clinical features such as markedly elevated fasting gastrin level (${\geq}$1,263 pg/mL) and findings from a CT scan and somatostatin receptor scan. Pathologic findings after the operation revealed malignant gastrinoma. He was confirmed to have parathyroid adenoma and MEN-1. Despite antisecretory therapy with proton pump inhibitors, an esophageal stricture developed, and we performed esophageal balloon dilatation and stent insertion.

56세 남자가 내원 2주 전부터 시작된 상복부 통증, 구토 그리고 설사를 주로로 내원하였으며 상부위장관 내시경 검사에서 역류성 식도염과 십이지장의 다발성 궤양을 보이고, 공복 혈청 가스트린 수치가 1,263 pg/mL 이상으로 정상의 10배 이상으로 상승되어 있어 Zollinger-Ellison 증후군으로 진단하였다. 복부 전산화단층촬영과 SRS를 통해 종양의 위치파악을 하고 수술적 절제를 하였으며, 부갑상선종이 동반되어 다발성내분비종 1형으로 진단하였다. 이후 PPI로 치료하였으나 식도 협착 소견이 반복되어 풍선확장술 및 식도 도관 삽입을 시행한 1예를 경험하여 보고한다.

Keywords

References

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