title: [A case of alpha-cell nesidioblastosis and hyperplasia with multiple glucagon-producing endocrine cell tumor of the pancreas].
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.
authors.affiliation: Departments of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Huapyong
authors.initials: H
authors.lastname: Kang
authors.affiliation: Departments of Internal Medicine, Pathology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Sewha
authors.initials: S
authors.lastname: Kim
authors.affiliation: Departments of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Tae Seop
authors.initials: TS
authors.lastname: Lim
authors.affiliation: Departments of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Hye Won
authors.initials: HW
authors.lastname: Lee
authors.affiliation: Departments of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Heun
authors.initials: H
authors.lastname: Choi
authors.affiliation: Departments of Internal Medicine, Surgery, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Chang Moo
authors.initials: CM
authors.lastname: Kang
authors.affiliation: Departments of Internal Medicine, Pathology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Ho Guen
authors.initials: HG
authors.lastname: Kim
authors.affiliation: Departments of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Seungmin
authors.initials: S
authors.lastname: Bang
mesh_terms: D000328:Adult; D053379:Chromogranin A / Q000097:blood; D005260:Female; D005934:Glucagon / Q000378:metabolism*; D050416:Glucagon-Secreting Cells / Q000378:metabolism; D006801:Humans; D006965:Hyperplasia / Q000150:complications / Q000175:diagnosis*; D007515:Islets of Langerhans / Q000000981:diagnostic imaging / Q000378:metabolism; D046768:Nesidioblastosis / Q000150:complications / Q000175:diagnosis*; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D010179:Pancreas / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography
chemical_list: D053379:Chromogranin A; D005934:Glucagon