Adult Onset Nesidioblastosis Treated by Subtotal Pancreatectomy
Abstract
Context Nesidioblastosis is a rare cause of non insulinoma pancreatogenous hypoglycemic syndrome seen in adults. It is characterized by postprandial hypoglycemia with high insulin and C-peptide levels without any detectable pancreatic lesion. The definitive diagnosis can be made only on histopathological examination of the resected specimen. Case report We report a case of a 50-year-old lady presenting with hypoglycemic attacks being misdiagnosed preoperatively as insulinoma and treated with enucleation leading to recurrence of symptoms after 6 months. Later medical therapy was tried which failed and patient needed subtotal pancreatectomy for resolution of symptoms. Conclusion Nesidioblastosis should be suspected in patients with endogenous hyperinsulinemic hypoglycemia without any detectable pancreatic tumor on preoperative imaging.
Image: Pancreatic ductal epithelium showing vacuolated neuroendocrine cells.
Downloads
References
Laidlaw GF. Nesidioblastoma, the islet cell tumor of the pancreas. Am J Pathol 1938; 14: 125-134.
Witteles RM, Straus II FH, Sugg SL, Koka MR, Costa EA, Kaplan EL. Adult-onset nesidioblastosis causing hypoglycemia: an important clinical entity and continuing treatment dilemma. Arch Surg 2001; 136: 656-663.
Qintar M, Sibai F, Taha M. Hypoglycemia due to adult onset nesidioblastosis, a diagnostic and management dileema. Avicenna J Med 2012;2:45-7
Service FJ, Natt N, Thompson GB, Grant CS, van Heerden JA, Andrews JC, et al. Noninsulinoma pancreatogenous hypoglycemia: a novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes. J Clin Endocrinol Metab 1999; 84:1582-9.
Cryer PE, Axelrod L, Grossman AB, Heller SR, Montori VM, Seaquist ER, et al. Evaluation and management of adult hypoglycemic disorders: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2009; 94:709.
Doppman JL, Chang R, Fraker DL, Norton JA; Alexander HR, Miller DL, et al. Localization of insulinomas to regions of the pancreas by intra-arterial stimulation with calcium. Ann Intern Med 1995; 123:269.
Kauhanen S, Seppanen M, Minn H, Gullichsen R, Salonen A, Alanen K, et al. Fluorine-18-L-Dihydroxyphenylalanine (18F-DOPA) Positron Emission Tomography as a Tool to Localize an Insulinoma or Cell Hyperplasia in Adult Patients. J Clin Endocrinol Metab 2007;92(4):1237–1244
Raffel A, Anlauf M, Hosch SB, Krausch M, Henopp T, Bauersfeld J, et al. Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes. World J Gastroenterol 2006; 12(44): 7221-7224.
Bright E, Garcea G, Ong SL, Madira W, Berry DP, Dennison AR. An Unusual Case of Concurrent Insulinoma and Nesidioblastosis. J Pancreas (Online) 2008; 9(5):649-653.
Heitz PU, Kloppel G, Hacki WH, Polak JM, Pearse AG. Nesidioblastosis: the pathologic basis of persistent hyperinsulinemic hypoglycemia in infants. Morphologic and quantitative analysis of seven cases based on specific immunostaining and electron microscopy. Diabetes 1977; 26: 632-642
Goossens A, Gepts W, Saudubray JM, Bonnefont JP, Nihoul-Fekete, Heitz PU, Kloppel G. Diffuse and focal nesidioblastosis. A clinicopathological study of 24 patients with persistent neonatal hyperinsulinemic hypoglycemia. Am J Surg Pathol 1989; 13:766-775
Arao T, Okada Y, Hirose A, Tanaka Y. A rare case of adult onset nesidioblastosis treated successfully with diazoxide. Endocr J 2006; 53: 95-100
Spanakis E, Gragnoli C. Successful medical management of status post-Roux-en-Y-gastric-bypass hyperinsulinemic hypoglycemia. Obes Surg 2009; 19:1333.
Draman MS, Ahern T, Smith T, O’Shea D. Selective intra-arterial calcium stimulation with hepatic venous sampling in investigation of hyperinsulinemic hypoglycemia. Ir J Med Sci 2006; 175(Suppl. 2):42.
Copyright (c) 2014 Rahul Amreesh Gupta, Roma Prahladbhai Patel, Sanjay Nagral
This work is licensed under a Creative Commons Attribution 4.0 International License.
As a member of Publisher International Linking Association, PILA, iMedPub Group’s JOP follows the Creative Commons Attribution License and Scholars Open Access publishing policies. Journal of the Pancreas is the Council Contributor Member of Council of Science Editors (CSE) and following the CSE slogan Education, Ethics, and Evidence for Editors.