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Open Access Case report

Diffuse nesidioblastosis with hypoglycemia mimicking an insulinoma: a case report

Chiara Ferrario1*, Delphine Stoll1, Ariane Boubaker2, Maurice Matter3, Pu Yan4 and Jardena J Puder1

Author Affiliations

1 Department of Endocrinology, University Hospital of Lausanne (CHUV), Rue du Bugnon 44, 1011, Lausanne, Switzerland

2 Department of Nuclear medicine, University Hospital of Lausanne (CHUV), Rue du Bugnon 44, 1011, Lausanne, Switzerland

3 Department of Surgery, University Hospital of Lausanne (CHUV), Rue du Bugnon 44, 1011, Lausanne, Switzerland

4 Department of Pathology, University Hospital of Lausanne (CHUV), Rue du Bugnon 25, 1011, Lausanne, Switzerland

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Journal of Medical Case Reports 2012, 6:332  doi:10.1186/1752-1947-6-332

Published: 2 October 2012

Abstract

Introduction

We describe a case of diffuse nesidioblastosis in an adult patient who presented with exclusively fasting symptoms and a focal pancreatic 111In-pentetreotide uptake mimicking an insulinoma.

Case presentation

A 23-year-old Caucasian man had severe daily fasting hypoglycemia with glucose levels below 2mmol/L. Besides rare neuroglycopenic symptoms (confusion, sleepiness), he was largely asymptomatic. His investigations revealed low venous plasma glucose levels, high insulin and C-peptide levels and a 72-hour fast test that were all highly suggestive for an insulinoma. Abdominal computed tomography and magnetic resonance imaging did not reveal any lesions. The sole imagery that was compatible with an insulinoma was a 111In-somatostatin receptor scintigraphy that showed a faint but definite focal tracer between the head and the body of the pancreas. However, this lesion could not be confirmed by endoscopic ultrasonography of the pancreas. Following duodenopancreatectomy, the histological findings were consistent with diffuse nesidioblastosis. Postoperatively, the patient continued to present with fasting hypoglycemia and was successfully treated with diazoxide.

Conclusion

In the absence of gastrointestinal surgery, nesidioblastosis is very rare in adults. In addition, nesidioblastosis is usually characterized by post-prandial hypoglycemia, whereas this patient presented with fasting hypoglycemia. This case also illustrates the risk for a false positive result of 111In-pentetreotide scintigraphy in the case of nesidioblastosis. Selective arterial calcium stimulation and venous sampling is the most reliable procedure for the positive diagnosis of insulinoma or nesidioblastosis and should be used to confirm any suspicion based on imaging modalities.

Keywords:
In-pentetreotide scintigraphy; Adult; Hypoglycemia; Imaging; Insulinoma; Nesidioblastosis