Recurrent hypoglycemia in a young female with nesidioblastosis: A case report and review of literature

Abstract

BACKGROUND

Nesidioblastosis is a rare condition involving abnormal proliferation of pancreatic β-cell, leading to excessive insulin production and severe hypoglycemia. In contrast to insulinomas, which are the most frequent cause of endogenous hyperinsulinism, nesidioblastosis lacks a definable mass, leaving the pancreas grossly unremarkable and invisible to conventional imaging techniques.

CASE SUMMARY

A 23-year-old female presented with a protracted 13-year history of recurrent hypoglycemia refractory to medical management (dextrose and diazoxide). Laboratory evaluation showed biochemical evidence of endogenous hyperinsulinism. Non-localizing conventional imaging was followed by a 68Ga-DOTATOC positron emission tomography/computed tomography scan, which demonstrated diffuse somatostatin-receptor uptake in the distal pancreas. The definitive diagnosis of nesidioblastosis was confirmed by histopathology following partial pancreatectomy.

CONCLUSION

This case highlights the diagnostic challenge associated with nesidioblastosis especially in resource-limited settings to enable early intervention.

Keywords: Nesidioblastosis; Hyperinsulinemic hypoglycemia; Insulinoma; Recurrent hypoglycemia; Functional B cell disorder; Case report

Core Tip: Adult-onset nesidioblastosis is an uncommon but important cause of endogenous hyperinsulinism that can mimic insulinoma and lead to years of misdiagnosis. This case illustrates how persistent hypoglycemia despite standard medical therapy should prompt consideration of rare etiologies and the use of advanced imaging, such as 68Ga-DOTATOC positron emission tomography/computed tomography, when conventional studies are non-localizing. Early recognition improves the likelihood of timely surgical management and prevents prolonged morbidity.