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一名患有持续性高胰岛素血症性低血糖症的成年人的胰腺成神经细胞瘤。

Nesidioblastosis of the pancreas in an adult with persistent hyperinsulinemic hypoglycemia.

作者信息

Albers N, Löhr M, Bogner U, Loy V, Klöppel G

机构信息

Institute of Pathology, University of Hamburg, Berlin, West Germany.

出版信息

Am J Clin Pathol. 1989 Mar;91(3):336-40. doi: 10.1093/ajcp/91.3.336.

DOI:10.1093/ajcp/91.3.336
PMID:2646913
Abstract

The rare finding of pancreatic nesidioblastosis in an adult is described. A 43-year-old woman presented with a two-year history of hypoglycemic hyperinsulinism. Extensive diagnostic procedures revealed no insulinoma. Subtotal (75%) pancreatectomy relieved her symptoms; she has normal insulin levels 2.5 years after surgery. The pancreatic specimen revealed only discrete islet cell abnormalities, namely B-cells budding off ductular epithelium, islets in apposition to ducts, slight islet cell hypertrophy, and islet enlargement. Immunohistochemistry showed normal total endocrine cell content as well as normal proportions of islet cell subpopulations. Review of 20 cases in the literature and the authors' experience led to subtotal (75-90%) pancreatectomy as the treatment of choice. The authors conclude that the pediatric disease of nesidioblastosis may rarely occur in adults and that the paucity of histologic findings makes the exclusion of an insulinoma mandatory.

摘要

本文描述了一例罕见的成人性胰岛母细胞增生症病例。一名43岁女性有两年低血糖性高胰岛素血症病史。广泛的诊断检查未发现胰岛素瘤。行胰腺次全切除术(切除75%)后症状缓解;术后2.5年胰岛素水平正常。胰腺标本仅显示散在的胰岛细胞异常,即B细胞从导管上皮出芽、胰岛与导管相邻、轻度胰岛细胞肥大以及胰岛增大。免疫组化显示内分泌细胞总量正常以及胰岛细胞亚群比例正常。回顾文献中的20例病例及作者的经验,得出胰腺次全切除术(切除75 - 90%)为首选治疗方法。作者得出结论,胰岛母细胞增生症这种儿科疾病在成人中可能罕见发生,且组织学表现缺乏使得排除胰岛素瘤成为必要。

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引用本文的文献

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Biomedicines. 2023 Jun 16;11(6):1732. doi: 10.3390/biomedicines11061732.
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Functional Localization of Adult-Onset Idiopathic Nesidioblastosis.成人起病型特发性胰岛细胞增殖症的功能定位
Case Rep Endocrinol. 2022 Oct 7;2022:2802975. doi: 10.1155/2022/2802975. eCollection 2022.
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A Unique Case of Mediastinal Teratoma with Mature Pancreatic Tissue, Nesidioblastosis, and Aberrant Islet Differentiation: a Case Report and Literature Review.
一例具有成熟胰腺组织、胰岛细胞增殖症和异常胰岛分化的纵隔畸胎瘤的独特病例:病例报告及文献综述
Endocr Pathol. 2016 Mar;27(1):21-4. doi: 10.1007/s12022-015-9393-4.
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