yperinsulinemic hypoglycemia caused by nesidioblastosis associated with non-surgical weight loss

Main Article Content

Natalia Awramiszyn-Fernandez http://orcid.org/0000-0003-2768-0846
José Luis Paz-Ibarra http://orcid.org/0000-0002-2851-3727
Jacsel Suárez-Rojas http://orcid.org/0000-0003-2377-7333
Cesar Eugenio Rodriguez-Alegría http://orcid.org/0000-0002-4733-6279
José René Somocurcio-Peralta http://orcid.org/0000-0002-5198-4113
Enrique Javier Ramírez-Delpino http://orcid.org/0000-0001-5251-7974
Alberto Alejandro Teruya-Gibu http://orcid.org/0000-0003-3816-4011

Keywords

Hyperinsulinism, Hypoglycemia, Nesidioblastosis

Abstract

Background: Nesidioblastosis is a rare disease in adults caused by pancreatic islet hyperplasia, producing hypoglycemia due to an increase in insulin production. The pathogenesis is poorly understood, however a clinical picture characterized by hypoglycemia with high levels of insulin and C-peptide without any detectable pancreatic lesion has been described, its final diagnosis is histological.


Case report: We report a case of a 36-year-old female patient who presents with recurrent episodes of hypoglycemia symptomatic predominantly postprandial, associated with insulin and C-peptide elevation. Images were made (triphasic computed tomography, magnetic resonance imaging, echoendoscopy and octreoscan), being negative for insulinoma. Subsequent tests included calcium stimulation of the pancreas, revealing marked insulin release in more than one pancreatic segment. The patient was subjected to distal pancreatectomy confirming in the histological study the diagnosis of nesidioblastosis.


Conclusions: Nesidioblastosis accounts for less than 5% of the cases of cases of hyperinsulinemic hypoglycemia in adult patients, should be considered as a differential diagnosis in patients with persistent hypoglycemia, the diagnosis is difficult and usually requires histological confirmation.

Abstract 229 | PDF (Spanish) Downloads 77 HTML (Spanish) Downloads 205

References

1. Davi MV, Pia A, Guarnotta V, Pizza G, Colao A, Faggiano A. The treatment of hyperinsulinemic hypoglycaemia in adults:an update. J Endocrinol Invest. 2017;40(1):9-20.

2. Ramírez-González LR, Sotelo-Álvarez JA, Rojas-Rubio P, Macías-Amezcua MD, Orozco-Rubio R, Fuentes-Orozco C. Nesidioblastosis en el adulto:reporte de un caso. Cir Cir. 2015;83(4):324-8.

3. Chen X, Kamel D, Barnett B, Yung E, Quinn A, Nguyen C. An unusual presentation of post gastric bypass hypoglycemia with both postprandial and fasting hypoglycemia. Endocrinol Diabetes Metab Case Rep. 2018;2018:18-0089.

4. Woo C-Y, Jeong JY, Jang JE, Leem J, Jung CH, Koh EH, et al. Clinical features and causes of endogenous hyperinsulinemic hypoglycemia in Korea. Diabetes Metab J. 2015;39(2):126.

5. Laidlaw GF. Nesidioblastoma, the islet tumor of the pancreas. Am J Pathol. 1938;14(2):125-35.

6. Sandler R, Horwitz DL, Rubenstein AH, Kuzuya H. Hypoglycemia and endogenous hyperinsulinism complicating diabetes mellitus. Application of the C-peptide assay to diagnosis and therapy. Am J Med. 1975;59:730-6.

7. Valente LG, Antwi K, Nicolas GP, Wild D, Christ E. Clinical presentation of 54 patients with endogenous hyperinsulinaemic hypoglycaemia:a neurological chameleon (observational study). Swiss Med Wkly. 2018;148:w14682. doi:10.4414/smw.2018.14682

8. Witteles RM. Adult-onset nesidioblastosis causing hypoglycemia:an important clinical entity and continuing treatment dilemma. Arch Surg. 2001;136(6):656.

9. De Santibañes M, Cristiano A, Mazza O, Grossenbacher L, de Santibañes E, Sánchez-ClariáR, et al. Síndrome de hipoglucemia por hiperinsulinismo endógeno:tratamiento quirúrgico. Cir Esp. 2014;92(8):547-52.

10. Grant CS. Insulinoma. Best Pract Res Clin Gastroenterol. 2005;19(5):783-98. doi:10.1016/j.bpg.2005.05.008

11. 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(3):709-28.

12. Kenney B, Tormey CA, Qin L, Sosa JA, Jain D, Neto A. Adult nesidioblastosis. Clinicopathologic correlation between pre-operative selective arterial calcium stimulation studies and post-operative pathologic findings. JOP. 2008;9(4):504-11.

13. Taha M, Qintar M, Sibai F. Hypoglycemia due to an adult-onset nesidioblastosis, a diagnostic and management dilemma. Avicenna J Med. 2012;2(2):45.

14. McLaughlin T, Peck M, Holst J, Deacon C. Reversible hyperinsulinemic hypoglycemia after gastric bypass:a consequence of altered nutrient delivery. J Clin Endocrinol Metab. 2010;95(4):1851-5.

15. Paz-Ibarra JL. Pruebas diagnósticas en endocrinología:de la clínica al laboratorio de hormonas e imágenes. España:Editorial Académica Española;2017.

16. Thompson SM, Vella A, Thompson GB, Rumilla KM, Service FJ, Grant CS, et al. Selective arterial calcium stimulation with hepatic venous sampling differentiates insulinoma from nesidioblastosis. J Clin Endocrinol Metab. 2015;100(11):4189-97.

17. Gupta RA, Patel RP, Nagral S. Adult onset nesidioblastosis treated by subtotal pancreatectomy. JOP. 2013;14(3):286-8.

18. Rumilla KM, Erickson LA, Service FJ, Vella A, Thompson GB, Grant CS, et al. Hyperinsulinemic hypoglycemia with nesidioblastosis:histologic features and growth factor expression. Mod Pathol. 2009;22(2):239-45.

19. Raffel A, Krausch MM, Anlauf M, Wieben D, Braunstein S, Klöppel G, et al. Diffuse nesidioblastosis as a cause of hyperinsulinemic hypoglycemia in adults:a diagnostic and therapeutic challenge. Surgery. 2007;141(2):179-84.

20. Gilis-Januszewska A, Piątkowski J, Skalniak A, Piwońska-Solska B, Nazim J, Pach D, et al. Noninsulinoma pancreatogenous hypoglycaemia in adults —a spotlight on its genetics. Endokrynol Pol. 2015;66(4):11.

21. Dardano A, Daniele G, Lupi R, Napoli N, Campani D, Boggi U, et al. Nesidioblastosis and insulinoma:a rare coexistence and a therapeutic challenge. Front Endocrinol (Lausanne). 2020;11:10. doi:10.3389/fendo.2020.00010

22. Dravecka I, Lazurova I. Nesidioblastosis in adults. Neoplasma. 2014;61(3):252-6. doi:10.4149/neo_2014_047

23. Tharakan G, Behary P, Wewer-Albrechtsen NJ, Chahal H, Kenkre J, D Miras A, et al. Roles of increased glycaemic variability, GLP-1 and glucagon in hypoglycaemia after roux-en-Y gastric bypass. Eur J Endocrinol. 2017;177(6):455-64. doi:10.1530/EJE-17-0446

24. Christ E, Wild D, Antwi K, Waser B, Fani M, Schwanda S, et al. Preoperative localization of adult nesidioblastosis using ⁶⁸Ga-DOTA-exendin-4-PET/CT. Endocrine. 2015;50(3):821-3. doi:10.1007/s12020-015-0633-7

25. Reubi JC, Perren A, Rehmann R, Waser B, Christ E, Callery M, et al. Glucagon-like peptide-1 (GLP-1) receptors are not overexpressed in pancreatic islets from patients with severe hyperinsulinaemic hypoglycaemia following gastric bypass. Diabetologia. 2010;53(12):2641-5. doi:10.1007/s00125-010-1901-y