A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

Carlos Hernández Brito1*, Mauricio Luis Barrón Soto2, David Ramírez Sosa3, Dante Díaz Martínez4, María Alicia Díaz Orea5, Arsenio Torres Delgado2, Eibar Guzmán Vera2, Héctor David Pérez Domínguez2, Pedro Arredondo Ruiz1

1Department of Surgical Oncology and Oncology, Millenium Medical Center, Veracruz, México. 2Department of Gastrointestinal and Laparoscopic Surgery, Hospital Puebla, Puebla, México.  3Department of Endocrinology, Hospital Puebla, Puebla, México. 4Department of Pathology, Hospital Puebla, Puebla, México. 5Departament of Experimental Immunology, Benemérita Universidad Autónoma de Puebla, Puebla, México.

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Introduction: Nesidioblastosis is a hyperplasia of the beta cells that causes persistent hyperinsulinemic hypoglycemia, unfortunately, it is difficult to diagnose through imaging studies.

Case presentation: A 68-year-old woman with palpitations, diaphoresis, dizziness and alterations in the level of consciousness that improve with food intake. Laboratories: glucose 35.0 mg/dl, insulin 12.5 mUI/ml, proinsulin 14.1 pmol/L and peptide C 2.55 ng/ml. Octreoscan reveals an abnormal epigastric uptake area and the tomography shows a focal hypervascular lesion in pancreatic body of 12×11 mm. A distal pancreatectomy was performed without palpating the tumor in transoperative period, and a diffuse nesiodioblastosis was reported in the histopathological study. The patient persists with hypoglycemia and an additional pancreatic resection was performed, resecting 90% of the organ. Although an insulinoma was not located in the piece, an area surrounded by connective tissue was observed where islet hyperplasia was more accentuated.

Discussion: The pathophysiology of nesidioblastosis is unknown, its main differential diagnosis is insulinoma and this should be suspected when the tumor can not be identified. Most agree that a pancreatectomy that involves 60-80% of the total organ can control glucose levels with low risk of diabetes and pancreatic insufficiency.

Conclusion: The nesidioblastosis should be suspected when a hyperinsulinemic hypoglycemia is difficult to control and when a tumor can not be identified. The extension of the pancreatectomy should be individualized and if an insulinoma is not localized and a nesidioblastosis is suspected, an intraoperative histopathological examination with frozen section evaluation for the margins could determine the extent of pancreatectomy.

Keywords: Nesidioblastosis; Insulinoma; Pancreatic tumor; Pancreatic neuroendocrine tumor; Hyperinsulinemia; Hyperinsulinemic hypoglycemia

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Carlos Hernández Brito, Mauricio Luis Barrón Soto, David Ramírez Sosa, Dante Díaz Martínez, María Alicia Díaz Orea, Arsenio Torres Delgado, Eibar Guzmán Vera, Héctor David Pérez Domínguez, Pedro Arredondo Ruiz. A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review. International Journal of Case Reports, 2020 4:169. DOI: 10.28933/ijcr-2020-10-0506


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