Adult Nesidioblastosis in Chronic Kidney Disease

Abstract

Context: Nesidioblastosis is a rare cause of hyperinsulinemic hypoglycemia in adults. The diagnosis is further complicated in patients with kidney failure, since impaired renal function can cause hypoglycemia by itself and diagnostic criteria for this clinical scenario have not been developed yet.

Case description: We present the case report of a 36-year-old patient with end stage chronic kidney disease who presented to the emergency department because of hypoglycemia. However, the patient's hypoglycemia did not respond well to medical treatment; the diagnosis of hyperinsulinemic hypoglycemia was made due to the presence of inappropriately high levels of insulin, proinsulin, and C-peptide during an episode of hypoglycemia. Imaging studies were performed without any conclusive findings; so selective intra-arterial pancreatic stimulation with hepatic venous sampling (SACTS) was done. Based on the results of this study the patient was referred for subtotal pancreatectomy. Classic criteria for the diagnosis of insulinoma with SACTS required a 2-fold increase in insulin levels but newer criteria suggest thresholds that are useful in the differential diagnosis of insulinoma and nesidioblastosis. In our patient, the former criteria were positive; however, the new criteria were not compatible with insulinoma but with nesidioblastosis, which was the final histopathological diagnosis.

Conclusion: This seems to be the first case report of a patient with end stage chronic kidney disease and nesidioblastosis, as well as the first case of hyperinsulinemic hypoglycemia in the context of kidney failure diagnosed by SACTS. We consider this method to be very useful in patients with renal impairment because peripancreatic insulin levels do not depend on the renal function.

Figure 1

Anatomical Relationship Between Areas and Arteries of the Pancreas Stimulated With Calcium. Relationship of the main arteries of the pancreas and the areas that irrigate: gastroduodenal (GDA), superior mesenteric (SMA), and splenic artery. The splenic artery was divided into proximal (PES) and distal (DES) to differentiate the tail of the body. The proper hepatic artery was included to rule out tumor activity in the liver (PHE). The numbers indicate the seconds at which the sample was taken after the calcium injection. This suggests the most likely localization of the lesions.

Figure 2

Hematoxylin and eosin stain of cell-block preparations from biopsy specimen. (a) Several clusters of β pancreatic cells are shown (arrows) (10x). (b) The typical cluster distribution near blood vessels is depicted (10x). (c) Pancreatic islets vary in size; very large islets are shown (arrow) (10x). (d) Hematoxylin and eosin stain shows cells with atypical morphology characterized by prominent nuclei and abundant granulated eosinophilic cytoplasm (40x).