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Cited by 14Neuroendocrine Tumor Research AdvancesPancreatic function and diabetesDiabetes and associated disorders

Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

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Dardano, Angela, Daniele, Giuseppe, Lupi, Roberto, Napoli, Niccolò, Campani, Daniela, Boggi, Ugo, Del Prato, Stefano, Miccoli, Roberto·2020·Frontiers in Endocrinology
DOI 10.3389/fendo.2020.00010PMC PMC6996476
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Abstract

Background: Nesidioblastosis and insulinoma are disorders of the endocrine pancreas causing endogenous hyperinsulinemic hypoglycemia. Their coexistence is very unusual and treatment represents a still unresolved dilemma. Case description: the patient was a 43-yr-old Caucasian woman, with a 2-year history of repeated severe hypoglycemic events. The diagnostic work-up was strongly suggestive of insulinoma and the patient was submitted to surgical treatment carried out laparoscopically under robotic assistance. However, surgical exploration and intraoperative ultrasonography failed to detect a pancreatic tumor. Resection was therefore carried out based on the results of selective intra-arterial calcium stimulation test, following a step-up approach, eventually leading to a pancreatoduodenectomy at the splenic artery. The histopathology examination and the immunohistochemical staining were consistent with adult onset nesidioblastosis. After surgery, the patient continued to experience hypoglycemia with futile response to medical treatments (octreotide, calcium antagonists, diazoxide, prednisone). Following multidisciplinary evaluation and critical review of a repeat abdominal computed tomography scan, a small nodular lesion was eventually identified in the tail of the pancreas. The nodule was enucleated laparoscopically and the pathological examination revealed an insulinoma. In spite of the insulinoma resection, glycemic values were only partially restored, with residual nocturnal hypoglycemia. Administration of uncooked cornstarch (1.25 g/kg body weight) at bedtime was associated with significant improvement of interstitial glucose levels (p<0.0001) and reduction of nocturnal hypoglycemia episodes (p=0.0002). Conclusions: This report describes a rare coexistence of adult onset nesiodioblastosis and insulinoma, suggesting the existence of a wide and continuous spectrum of proliferative β cell changes. Moreover, we propose that uncooked cornstarch may offer an additional approach to alleviate the hypoglycemic episodes when surgery is impracticable/unaccepted.

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  • The potassium channel activator diazoxide has been shown to alleviate symptoms in some cases of NIPHS.

    “While a low carbohydrate diet or α -glucosidase inhibitors are not effective in most patients, the potassium channel activator diazoxide has been shown to alleviate symptoms in some cases [58,59].”

    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 [59]

    The Discussion (page unavailable) lists diazoxide among treatments reported in isolated nesidioblastosis case reports, supporting its use in some cases. It does not say that diazoxide alleviated symptoms in those cases; in the paper’s own Case Report, it was withdrawn because of adverse effects. (LLM, conf=0.82)

    Experimental — accuracy not yet measured

  • A low-carbohydrate diet or α-glucosidase inhibitors are not effective in most patients with NIPHS.

    “While a low carbohydrate diet or α -glucosidase inhibitors are not effective in most patients, the potassium channel activator diazoxide has been shown to alleviate symptoms in some cases [58,59].”

    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 [59]

    The Discussion lists a low-carbohydrate diet as a treatment option for nesidioblastosis but does not assess its effectiveness in most patients. It says common medical therapy was ineffective or caused side effects in one patient; the passages found do not address α-glucosidase inhibitors or establish the claimed outcome across patients (searched: low-carbohydrate diet, α-glucosidase inhibitors, acarbose). (LLM, conf=0.76)

    Experimental — accuracy not yet measured

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Cited by

Sorted by citations

Authors
Dardano, A., Daniele, G., Lupi, R., Napoli, N., Campani, D., Boggi, U., Del Prato, S., & Miccoli, R.
Affiliations
University of Pisa (IT)
Corresponding author
Dardano, Angela
ORCID
Venue
Frontiers in Endocrinology
Publisher
Frontiers Media SA
Published
2020-01-24
Volume
11
Pages
10-10
ISSN
1664-2392 (ISSN-L)
DOI
10.3389/fendo.2020.00010
PMC
PMC6996476
OpenAlex
W3002489426
Cited by
14
References
8
Citations by year
2024: 5 · 2023: 4 · 2022: 3 · 2021: 1 · 2020: 1
FWCI
1.61
Type
journal article
Language
en
Open access
Licence
Locations
  • Frontiers in Endocrinology (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion, cc-by)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Topics
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
Keywords
nesidioblastosis, insulinoma, hyperinsulinemic hypoglycemia, pancreatic beta-cell proliferation, nocturnal hypoglycemia, uncooked cornstarch, pancreaticoduodenectomy, intraoperative ultrasound, octreotide, diazoxide, prednisone
MeSH terms
Adult, Blood Glucose, Circadian Rhythm, Diagnosis, Differential, Female, Humans, Hypoglycemia, Insulinoma, Pancreatectomy, Pancreatic Neoplasms, Starch, Congenital Hyperinsulinism, Nesidioblastosis

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