Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia

De Sousa, Sunita M.C.; Haghighi, Koroush S.; Qiu, Min Ru; Greenfield, Jerry R.; Chen, Daniel L.T.

doi:10.1097/mpa.0000000000000430

Cited by 13Neuroendocrine Tumor Research AdvancesPancreatic and Hepatic Oncology ResearchPancreatic function and diabetes

Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia

De Sousa, Sunita M.C., Haghighi, Koroush S., Qiu, Min Ru, Greenfield, Jerry R., Chen, Daniel L.T.·2016·Pancreas

DOI 10.1097/mpa.0000000000000430

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Abstract

Herein, we report the first case of concomitant nesidioblastosis, pancreatic neuroendocrine tumor, and intraductal papillary mucinous neoplasia. The combination is significant as each of these pathological entities is independently very rare. The patient was a 33-year-old man who presented with symptomatic hyperinsulinemic hypoglycemia and no risk factors for pancreatic disease. Abdominal imaging showed an isolated 12 mm pancreatic lesion, whilst selective arterial calcium stimulation testing demonstrated multiple territories of insulin excess. He proceeded to subtotal pancreatectomy. Histopathology revealed an endocrine microadenoma, α and β cell nesidioblastosis, and multifocal intraductal papillary mucinous neoplasia. The endocrine microadenoma and nesidioblastosis stained for insulin, suggesting both likely contributed to hypoglycemia. Glucagon immunohistochemistry was also positive, though there were no clinical features of glucagon excess. Hypoglycemia resolved postoperatively. This case and other evidence from the literature suggest that hyperplasia and neoplasia may occur sequentially in the pancreas, and that endocrine and exocrine tumorigenesis may be linked in some individuals. Further study is required to identify a unifying mechanism, and to elucidate potential ramifications in the management of patients with pancreatic neoplasms.

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Some NIPHS patients are relieved from all symptoms upon left-sided partial/subtotal pancreatectomy, while others need total pancreatectomy for symptom control.
“While some patients are relieved from all symptoms upon left-sided partial/subtotal pancreatectomy, others need to undergo total pancreatectomy for symptom control [63-65].”
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Lopes, A. A., Miranda, A. C. G., Maior, M. D. R. M. S., de Mello, R. V., & Bandeira, F. A. F. (2020) · American Journal of Case Reports · doi:10.12659/ajcr.922778 · Cited by 11 · see citing works

Authors: De Sousa, S. M. C., Haghighi, K. S., Qiu, M. R., Greenfield, J. R., & Chen, D. L. T.
Affiliations: UNSW Sydney (AU)
St Vincent's Hospital Melbourne (AU)
Garvan Institute of Medical Research (AU)
Corresponding author: Greenfield, Jerry R.
Chen, Daniel L.T.
ORCID: Haghighi, Koroush S.
Qiu, Min Ru
Greenfield, Jerry R.
Venue: Pancreas
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 2016-01
Volume: 45
Issue: 1
Pages: 154-159
ISSN: 0885-3177 (ISSN-L)
DOI: 10.1097/mpa.0000000000000430
OpenAlex: W2411962594
Cited by: 13
References: 31
Citations by year: 2025: 1 · 2024: 2 · 2023: 2 · 2022: 1 · 2020: 3 · 2019: 1 · 2018: 1 · 2017: 1 · 2016: 1
FWCI: 0.79
Type: journal article
Language: en
Open access: closed
Locations: Pancreas (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
Health Sciences › Medicine › Oncology › Pancreatic and Hepatic Oncology Research
Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
Keywords: nesidioblastosis, hyperinsulinemic hypoglycemia, pancreatic neuroendocrine tumors, subtotal pancreatectomy, insulin excess, pancreatic neoplasia
MeSH terms: Adenoma, Adult, Biopsy, Blood Glucose, Diagnosis, Differential, Humans, Hyperinsulinism, Hypoglycemia, Immunohistochemistry, Insulin, Male, Neoplasms, Multiple Primary, Pancreatic Function Tests, Pancreatic Neoplasms, Predictive Value of Tests, Risk Factors, Time Factors, Tomography, X-Ray Computed, Treatment Outcome, Neoplasms, Cystic, Mucinous, and Serous, Neuroendocrine Tumors, Nesidioblastosis

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Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia

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