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다발성 글루카곤 생성 내분비세포종을 동반한 췌장 알파세포의 Nesidioblastosis 및 과다증식 1예
A Case of Alpha-cell Nesidioblastosis and Hyperplasia with Multiple Glucagon-producing Endocrine Cell Tumor of the Pancreas

대한소화기학회지 2014년 63권 4호 p.253 ~ p.257

강화평(Kang Hua-Pyong) - 연세대학교 의과대학 내과학교실
김세화(Kim Se-Wha) - 연세대학교 의과대학 병리학교실
임태섭(Lim Tae-Seop) - 연세대학교 의과대학 내과학교실
이혜원(Lee Hye-Won) - 연세대학교 의과대학 내과학교실
최흔(Choi Heun) - 연세대학교 의과대학 내과학교실
강창무(Kang Chang-Moo) - 연세대학교 의과대학 외과학교실
김호근(Kim Ho-Guen) - 연세대학교 의과대학 병리학교실
방승민(Bang Seung-Min) - 연세대학교 의과대학 내과학교실

Abstract

Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells. It also means maldistribution of islet cells within the ductules of exocrine pancreas. Generally, nesidioblastosis occurs in beta-cell and causes neonatal hyperinsulinemic hypoglycemia or adult noninsulinoma pancreatogenous hypoglycemia syndrome. Alpha-cell nesidioblastosis and hyperplasia is an extremely rare disorder. It often accompanies glucagon-producing marco- and mircoadenoma without typical glucagonoma syndrome. A 35-year-old female was referred to our hospital with recurrent acute pancreatitis. On radiologic studies, 1.5 cm sized mass was noted in pancreas tail. Cytological evaluation with EUS-fine-needle aspiration suggested serous cystadenoma. She received distal pancreatectomy. The histologic examination revealed a 1.7 cm sized neuroendocrine tumor positive for immunohistochemical staining with glucagon antibody. Multiple glucagon-producing micro endocrine cell tumors were scattered next to the main tumor. Additionally, diffuse hyperplasia of pancreatic islets and ectopic proliferation of islet cells in centroacinar area, findings compatible to nesidioblastosis, were seen. These hyperplasia and almost all nesidioblastic cells were positive for glucagon immunochemistry. Even though serum glucagon level still remained higher than the reference value, she has been followed-up without any evidence of recurrence or hormone related symptoms. Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review.

키워드

Alpha cell, Nesidioblastosis, Hyperplasia, Glucagon, Neuroendocrine tumor

KMID : 0614620140630040253

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ICD 03

건강보험코드

다발성 글루카곤 생성 내분비세포종을 동반한 췌장 알파세포의 Nesidioblastosis 및 과다증식 1예 A Case of Alpha-cell Nesidioblastosis and Hyperplasia with Multiple Glucagon-producing Endocrine Cell Tumor of the Pancreas

Abstract

키워드

다발성 글루카곤 생성 내분비세포종을 동반한 췌장 알파세포의 Nesidioblastosis 및 과다증식 1예
A Case of Alpha-cell Nesidioblastosis and Hyperplasia with Multiple Glucagon-producing Endocrine Cell Tumor of the Pancreas