Successful Medical Treatment of Adult Nesidioblastosis With Pasireotide over 3 Years: A Case Report
Open accessAbstract
Nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Diagnosis is often challenging and therapeutic options are scarce.In 2009, a 46-year-old female patient presented with recurrent severe hypoglycemia and immediate recovery after glucose ingestion. Although 72-h-fasting test was positive, various imaging technologies (sonography, computed tomography, somatostatin receptor scintigraphy, dopamine receptor positron emission tomography [DOPA-PET]) were negative. Endoscopic ultrasound revealed a lesion in the pancreatic corpus, whereas selective arterial calcium stimulation test, portal venous sampling and GLP-1-receptor scintigraphy were indicative of a lesion in the pancreatic tail, which was surgically removed. The histopathologic examination revealed beta cell hyperplasia and microadenomas expressing glucagon. After surgery, the patient was free of symptoms for 6 months, after which hypoglycemic episodes recurred. After unsuccessful treatment with corticosteroids and somatostatin analogs, treatment with pasireotide, a novel somatostatin analog with high affinity to somatostatin receptor 5 and a possible side effect of hyperglycemia, was initiated (0.6 mg BID). To date, our patient has been free of severe hypoglycemic episodes ever since. Yearly repeated imaging procedures have shown no abnormities over the last 3 years.We report for the first time that pasireotide was successfully used in the treatment of adult nesidioblastosis.
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Because somatostatin analogues also inhibit glucagon secretion, symptoms of hypoglycemia may occur even more frequently in a subset of NIPHS patients.
“Since these substances also inhibit the secretion of glucagon, symptoms of hypoglycemia may occur even more frequently in a subset of patients [52,61,62].”
From An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS) (2023) · cites this paper as [62]
The Discussion (page unavailable) states that glucagon inhibition is mediated mainly by somatostatin receptor 2, supporting the claim that somatostatin analogues can inhibit glucagon secretion. It does not report more frequent hypoglycemic symptoms in a subset of NIPHS patients; instead, it describes avoidance of severe hypoglycemia with pasireotide. (LLM, conf=0.86)
Experimental — accuracy not yet measuredStimulation of somatostatin receptors by octreotide, lanreotide, or pasireotide has repeatedly been described in the literature in the context of NIPHS.
“The stimulation of somatostatin receptors by octreotide, lanreotide, or pasireotide has also repeatedly been described in the literature in the context of NIPHS.”
From An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS) (2023) · cites this paper as [62]
The Introduction and Clinical Case (page not available) report pasireotide treatment for adult nesidioblastosis and an unsuccessful trial of octreotide. The Discussion also compares pasireotide with octreotide in a reported post-bypass hypoglycemia case. Our copy does not mention lanreotide or establish that all three drugs have repeatedly been described in the NIPHS literature. (LLM, conf=0.87)
Experimental — accuracy not yet measuredInsulin secretion is reduced by somatostatin receptor type 2 and 5 signaling, while glucagon secretion is mainly inhibited by receptor type 2 signaling.
“Insulin secretion is reduced by somatostatin receptor type 2 and 5 signaling, while glucagon secretion is mainly inhibited by receptor type 2 signaling.”
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- Authors
- Schwetz, V., Horvath, K., Kump, P., Lackner, C., Perren, A., Forrer, F., Pieber, T. R., Treiber, G., Sourij, H., & Mader, J. K.
- Affiliations
- University of Bern (CH)
- Corresponding author
- Sourij, Harald
- ORCID
- Venue
- Medicine
- Published
- 2016-04
- Volume
- 95
- Issue
- 14
- Pages
- e3272
- ISSN
- 0025-7974 (ISSN-L)
- PMC
- PMC4998801
- OpenAlex
- W2335966923
- Cited by
- 25
- References
- 23
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 4 · 2023: 5 · 2022: 3 · 2021: 2 · 2020: 2 · 2019: 2 · 2018: 3 · 2016: 1
- FWCI
- 1.88
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by-nd
- Locations
- Medicine (publishedVersion, cc-by-nd)
- PubMed Central (publishedVersion, cc-by-nd)
- PubMed (publishedVersion)
- Bern Open Repository and Information System (University of Bern) (submittedVersion, cc-by-nd)
- Open Access CRIS of the University of Bern
- Topics
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Keywords
- nesidioblastosis, pasireotide, somatostatin analogues, endogenous hyperinsulinemic hypoglycemia, pancreatic beta-cell hyperplasia, somatostatin receptor 5, somatostatin receptor scintigraphy, endoscopic ultrasound, recurrent hypoglycemia
- MeSH terms
- Female, Humans, Middle Aged, Remission Induction, Somatostatin, Time Factors, Nesidioblastosis
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