A rare case of non-insulinoma pancreatic hypoglycaemia syndrome (niphs) in an adult due to localised islet cell hyperplasia-successfully managed by enucleation

Nayak, Hemanta Kumar; Sothwal, Arpit; Raizaida, Nishant; Daga, Mradul Kumar; Agarwal, Anil; Durga, Garima

doi:10.1136/bcr.07.2011.4554

Cited by 2 publications

(2 citation statements)

References 13 publications

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“…Although it is a very useful test, in many hospitals it is not available. The major diagnostic histopathological criteria of nesidioblastosis include the exclusion of an insulinoma by macroscopic, microscopic and immunohistochemical examination, the presence of multiple beta cells with an enlarged and hyperchromatic nucleus and abundant clear cytoplasm, islets with normal spatial distribution of the various cell types, and no proliferative activity of endocrine cells [3,4,7,22] . As minor criteria have been described the presence of elongated islets, the increase in the number of islets, islets with lobular structure, and macronucleolos in beta cells [23] .…”

Section: Discussionmentioning

confidence: 99%

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

2020

IJCR

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Introduction: Nesidioblastosis is a hyperplasia of the beta cells that causes persistent hyperinsulinemic hypoglycemia, unfortunately, it is difficult to diagnose through imaging studies. Case presentation: A 68-year-old woman with palpitations, diaphoresis, dizziness and alterations in the level of consciousness that improve with food intake. Laboratories: glucose 35.0 mg/dl, insulin 12.5 mUI/ml, proinsulin 14.1 pmol/L and peptide C 2.55 ng/ml. Octreoscan reveals an abnormal epigastric uptake area and the tomography shows a focal hypervascular lesion in pancreatic body of 12×11 mm. A distal pancreatectomy was performed without palpating the tumor in transoperative period, and a diffuse nesiodioblastosis was reported in the histopathological study. The patient persists with hypoglycemia and an additional pancreatic resection was performed, resecting 90% of the organ. Although an insulinoma was not located in the piece, an area surrounded by connective tissue was observed where islet hyperplasia was more accentuated. Discussion: The pathophysiology of nesidioblastosis is unknown, its main differential diagnosis is insulinoma and this should be suspected when the tumor can not be identified. Most agree that a pancreatectomy that involves 60-80% of the total organ can control glucose levels with low risk of diabetes and pancreatic insufficiency. Conclusion: The nesidioblastosis should be suspected when a hyperinsulinemic hypoglycemia is difficult to control and when a tumor can not be identified. The extension of the pancreatectomy should be individualized and if an insulinoma is not localized and a nesidioblastosis is suspected, an intraoperative histopathological examination with frozen section evaluation for the margins could determine the extent of pancreatectomy.

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Section: Discussionmentioning

confidence: 99%

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

2020

IJCR

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“…Another important point is the differentiation of diffuse and focal adult nesidioblastosis. While focal forms of hyperinsulinemic hypoglycemia account for roughly 40% of cases in children/infants, focal adult nesidioblastosis is extremely rare, and only 10 cases have been described in the medical literature [ 12 , 55 , 111 , 151 , 159 , 203 , 210 , 225 , 250 ]. All other forms are described as diffuse, adult-onset nesidioblastosis since the morphological findings are distributed throughout (nearly) the entire pancreas.…”

Section: History Histopathological and Clinical Definition Of Hyperin...mentioning

confidence: 99%

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia

Dieterle,

Husari,

Prozmann

et al. 2023

Biomedicines

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Differential diagnosis of hypoglycemia in the non-diabetic adult patient is complex and comprises various diseases, including endogenous hyperinsulinism caused by functional β-cell disorders. The latter is also designated as nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). Clinically, this rare disease presents with unspecific adrenergic and neuroglycopenic symptoms and is, therefore, often overlooked. A combination of careful clinical assessment, oral glucose tolerance testing, 72 h fasting, sectional and functional imaging, and invasive insulin measurements can lead to the correct diagnosis. Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective. Therefore, nearly all patients currently undergo surgical resection of parts or the entire pancreas. Consequently, apart from faster diagnosis, more elaborate and less invasive treatment options are needed to relieve the patients from the dangerous and devastating symptoms. Based on a case of a 23-year-old man presenting with this disease in our department, we performed an extensive review of the medical literature dealing with this condition and herein presented a comprehensive discussion of this interesting disease, including all aspects from epidemiology to therapy.

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A rare case of non-insulinoma pancreatic hypoglycaemia syndrome (niphs) in an adult due to localised islet cell hyperplasia-successfully managed by enucleation

Cited by 2 publications

References 13 publications

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia

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