A Case Report of Insulinoma Relapse on Background Nesidioblastosis: A Rare Cause of Adult Hypoglycemia
Open accessAbstract
Context: Nesidioblastosis is a rare cause of adult hypoglycemia. Current medical therapy can mitigate disease symptoms. However, side effects and limited efficacy may prevent long-term disease management. Case Description: A 63-year-old white woman presented at our institution on April 2017 with a history of distal spleno-pancreatectomy for well-differentiated insulinoma in 2013. Hypoglycemic events did not resolve after surgery, and residual nesidioblastosis near the pancreatic resection margins was identified. Hypoglycemic episodes increased in frequency and severity despite high-dose diazoxide (DZX) therapy. On April 2016, octreotide was introduced but soon discontinued for inefficacy. When the patient arrived at our attention, add-on pasireotide was started and glucose levels monitored by subcutaneous sensor. Compared with DZX, 225 mg/d alone, sensor glucose during pasireotide + DZX 75 mg/d showed occurrence of severe hypoglycemia. Pasireotide was discontinued, and the instrumental workup (68Ga-DOTATOC CT/positron emission tomography, 99mTc-nanocolloid scintigraphy and echo-endoscopy + fine-needle aspiration biopsy) identified an insulinoma relapse. Subtotal pancreatectomy was performed without further recurrence of hypoglycemia over 9 months of follow-up. Conclusions: Although insulinoma relapses on background nesidioblastosis rarely occur, they should be considered as an alternate diagnosis when medical therapy fails to prevent hypoglycemia. Further studies are warranted to test whether the immunophenotypic signature of nesidioblastosis/insulinoma may provide insights for a tailored use of pasireotide.
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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 [61]
The Discussion says octreotide and lanreotide inhibit glucagon secretion, potentially impairing the response to hypoglycemia. It also says they may worsen the frequency of hypoglycemic episodes in insulinomas lacking SSTR2. That finding concerns a subset of insulinoma patients, not specifically a subset of patients with NIPHS. (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 [61]
The paper describes octreotide and lanreotide as somatostatin-receptor ligands used for refractory nesidioblastosis (Abstract), and discusses pasireotide as a multi-receptor ligand used in this case (Discussion; Conclusions). But it identifies only at least one earlier case of pasireotide use in adult nesidioblastosis (Abstract), so it does not establish that use of all three drugs in this context has repeatedly been described in the literature. (LLM, conf=0.82)
Experimental — accuracy not yet measured
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Cites
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Dieterle, M. P., Husari, A., Prozmann, S. N., Wiethoff, H., Stenzinger, A., Röhrich, M., Pfeiffer, U., Kießling, W. R., Engel, H., Sourij, H., et al. (2023) · Biomedicines · doi:10.3390/biomedicines11061741 · Cited by 5 · see citing works
“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].”
Background
- Authors
- Dauriz, M., Maneschi, C., Castelli, C., Tomezzoli, A., Fuini, A., Landoni, L., Malleo, G., Ferdeghini, M., Bonora, E., & Moghetti, P.
- Affiliations
- University of Verona (IT)
- Corresponding author
- Dauriz, Marco
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2018-12-28
- Volume
- 104
- Issue
- 3
- Pages
- 773-778
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2907579143
- Cited by
- 8
- References
- 2
- Citations by year
- 2025: 1 · 2023: 3 · 2020: 1 · 2019: 3
- FWCI
- 0.78
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Università degli Studi di Verona
- Topics
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Keywords
- nesidioblastosis, pasireotide, diazoxide, octreotide, subtotal pancreatectomy, somatostatin analogue therapy, 68Ga-DOTATOC PET/CT, fine-needle aspiration, distal pancreatectomy
- MeSH terms
- Diazoxide, Female, Humans, Hypoglycemia, Insulinoma, Middle Aged, Neoplasm Recurrence, Local, Pancreas, Pancreatectomy, Pancreatic Neoplasms, Somatostatin, Splenectomy, Treatment Outcome, Nesidioblastosis
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