Successful Control of Hypoglycemia with Pasireotide LAR in a Patient with Inappropriate Insulin Secretion

Abstract

Introduction Inappropriate insulin secretion could be due to several diseases. Nesidioblastosis is characterized by diffuse hyperplasia of pancreatic beta cells, causing organic hypoglycemia. No pancreatic lesions are found on the imaging of patients with this condition. Diazoxide is used as a first-line treatment but can be poorly tolerated because of its side effects, and therapeutic failure is possible. Somatostatin analogues have limited efficacy because of their poor affinity to somatostatin (SST) receptors. Pasireotide is a somatostatin analogue with a much higher affinity to SST receptors, especially SST5, and it could thus be more efficient for treating nesidioblastosis-related hypoglycemia. Observation A 56 years-old diabetic woman had symptoms of hypoglycemia, persistent after treatment’s withdrawal. A fasting test authentify an organic hypoglycemia, at 34mg/dL, a plasma insulin level at 6mUI/L above the 5 mU/L threshold, a C-peptide level at 1.9 ng/mL above the threshold of 0.6, and an insulin/C-peptide ratio 0.066, below the threshold of 1. No lesions were found on CT-scan or endoscopic ultrasound. Somatostatin receptor scintigraphy was also negative. Diazoxide and octreotide failed to improve the recurrence of hypoglycemia episodes. With pasireotide LAR, hypoglycemia disappeared and glycemia increased. Hyperglycemia was controlled with sitagliptin. The patient has now been treated with pasireotide LAR for two years, with no more episode of hypoglycemia until now. Discussion We present the first case of nesidioblastosis treatment with pasireotide LAR, with success. Patients diagnosed with nesidioblastosis and diazoxide-resistant hypoglycemia, or who experience difficulties with other treatments, could use pasireotide LAR in conjunction with glycemia monitoring, particularly if they are diabetic.

Adult focal β-cell nesidioblastosis: A case report

“…Kato et al [ 17 ] reported a case of adult-onset nesidioblastosis which was successfully treated with pancreaticoduodenectomy and octreotide, but their poor affinity to somatostatin receptors resulting in reduced efficiency. Pasireotide is another somatostatin analogue with a higher affinity to somatostatin receptors, and its long-acting release has been proved effective in isolated nesidioblastosis[ 18 ]. Other medications include glucocorticoids and calcium channel-blocking agents.…”

Adult focal β-cell nesidioblastosis: A case report and literature review

“…Rina Kato et al reported a case of adult-onset nesidioblastosis which was successfully treated with pancreaticoduodenectomy and octreotide [17] , but their poor affinity to somatostatin receptors resulting in reduced efficiency. Pasireotide is another somatostatin analogue with a higher affinity to somatostatin receptors, and its long-acting release has been proved effective in isolated nesidioblastosis [18] .…”

Pasireotide—a novel somatostatin receptor ligand after 20 years of use

“…Prostate cancer, colorectal cancer, pancreatic cancer, bladder cancer, and breast cancer have been reported to have a limited expression of SSTR1-4 and an increased expression of SSTR5, which may suggest potential benefits from the use of PAS in these diseases. [7,[98][99][100]. A study on the use of PAS in malignant melanoma (NCT01652547) has been recently completed; however, its results have not yet been published.…”