Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

Dardano, Angela; Daniele, Giuseppe; Lupi, R; Napoli, Niccolò; Campani, Daniela; Boggi, Ugo; Prato, Stefano Del; Miccoli, Roberto

doi:10.3389/fendo.2020.00010

Cited by 17 publications

(6 citation statements)

References 42 publications

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“…Treatment of nesidioblastosis is not always simple, it is not only dependent on medication and surgery, but also requires dietary control, dividing food intake into five to six daily meals, slowing gastric emptying, using fructose and avoiding stress at meals. Uncooked cornstarch is a source of slow release carbohydrates, it can produce low glucose peaks and maintains a stable blood glucose level during fasting; therefore, uncooked cornstarch can be considered in patients with persistent hypoglycemia particularly when surgery is impracticable/unaccepted and pharmacologic therapy is ineffective[ 20 ].…”

Section: Discussionmentioning

confidence: 99%

Adult focal β-cell nesidioblastosis: A case report

Tu¹,

Zhao²,

Gu³

2023

World J Clin Cases

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BACKGROUND Nesidioblastosis usually refers to a series of clinical manifestations caused by the proliferation of β-cells in pancreatic islets, and these clinical manifestations are hyperinsulinemia and persistent hypoglycemia. According to the size of the lesion, nesidioblastosis is divided into focal nesidioblastosis, diffuse nesidioblastosis and atypical nesidioblastosis, and its pathogenesis is still unclear. Nesidioblastosis is mainly seen in infants and rarely reported in adults, especially focal nesidioblastosis, which is difficult to distinguish from insulinoma. CASE SUMMARY We report a case of adult focal β-cell nesidioblastosis in which the preoperative diagnosis was insulinoma. The patient was a 48-year-old male who suffered from repeated morning and fasting palpitations, sweating, and severe disturbance of consciousness for 5 years. His blood glucose was found to be as low as 1.79 mmol/L during an attack. However, abdominal computed tomography showed no abnormalities. Magnetic resonance imaging and endoscopic ultrasonography demonstrated a nodular mass in the head of the pancreas, combined with hyperinsulinemia and high serum C-peptide. The patient was diagnosed with insulinoma and underwent Beger surgery; however, the postoperative pathological results showed nesidioblastosis. CONCLUSION Although surgical resection is the preferred option for nesidioblastosis, some cases can be treated non-surgically. In order to increase clinicians' understanding of nesidioblastosis, it is necessary to review the pathogenesis, diagnosis and treatment of this disease.

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Section: Discussionmentioning

confidence: 99%

Adult focal β-cell nesidioblastosis: A case report

Tu¹,

Zhao²,

Gu³

2023

World J Clin Cases

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“…Once the diagnosis of nesidioblastosis/NIPHS is suspected, treatment options need to be discussed with the patient. 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 ]. However, severe adverse effects have been reported in this context, which is also illustrated by the presented case.…”

Section: Discussionmentioning

confidence: 99%

An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS)

Dieterle,

Husari,

Prozmann

et al. 2023

Biomedicines

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Neurovegetative and autonomic symptoms are common presentations of various diseases, ranging from psychosomatic to severe organic disorders. A 23-year-old man presented with a history of recurrent presyncope, dizziness, and tachycardia. Repeated diagnostic work-up in various clinical settings could not identify any definite cause for approximately eight years. However, the incidental detection of postprandial and exercise-induced hypoglycemia was suggestive of an insulin-related disorder. A 72 h plasma glucose fasting test revealed endogenous hyperinsulinism. Upon imaging studies, no tumor mass potentially indicating insulinoma could be detected. 68Ga-DOTA-Exendin-4 PET/CT showed diffuse tracer enrichment throughout the whole pancreas. A subtotal pancreatectomy was performed, and the diagnosis of diffuse, adult-onset nesidioblastosis was established histopathologically. This corresponds to the clinical findings of a functional β-cell disorder, also known as non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). After nine months, the symptoms recurred, making complete pancreatectomy necessary. Postoperative laboratory evaluation exhibited no residual endogenous C-peptide production. This case illustrates the diagnostic challenges in patients presenting with unspecific, neurovegetative and autonomic symptoms with a severe and rare underlying cause.

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“…The case report revealed the potential efficacy of RCS on elevation of interstitial glucose levels and reduction of nocturnal hypoglycemia episodes in insulinoma patients, albeit has not been further validated [3] . Actually, RCS has been demonstrated to be efficacious and safe for hypoglycemia caused by other clinical conditions.…”

Section: Introductionmentioning

confidence: 90%

“…Currently, the general principle of nutritional intervention is the regular intake of complex carbohydrate-rich meals to control hypoglycemic episodes. An isolated case report depicted a patient with intractable hypoglycemia caused by a combination of nesidioblastosis and insulinoma, whose hypoglycemia was alleviated after bedtime supplementation with raw corn starch (RCS) [3] .…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Raw Corn Starch in Insulinoma-Related Hypoglycemia: A Promising Supportive Therapy

Li,

Chen,

Xiao

et al. 2024

Chinese Medical Sciences Journal

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Objective To investigate the efficacy of raw corn starch (RCS) in clinical management of insulinoma-induced hypoglycemia. MethodsWe retrospectively collected clinical data of insulinoma patients who received RCSsupplemented diet preoperatively, and analyzed the therapeutic effects of the RCS intervention on blood glucose control, weight change, and its adverse events. ResultsThe study population consisted of 24 case of insulinoma patients, 7 males and 17 females, aged 46.08 ± 14.15 years. Before RCS-supplemented diet, all patients had frequent hypoglycemic episodes (2.51 ± 3.88 times/week), concurrent with neuroglycopenia (in 83.3% of patients) and autonomic manifestations (in 75.0% of patients), with the median fasting blood glucose (FBG) of 2.70 [interquartile range (IQR): 2.50-2.90] mmol/L. The patients' weight increased by 0.38 (IQR: 0.05-0.65) kg per month, with 8 (33.3%) cases developing overweight and 7 (29.2%) cases developing obesity. All patients maintained the RCS-supplemented diet until they underwent tumor resection (23 cases) and transarterial chemoembolization for liver metastases (1 case). For 19 patients receiving RCS throughout the day, the median FBG within one week of nutritional management was 4.30 (IQR: 3.30-5.70) mmol/L, which was a significant increase compared to prenutritional level [2.25 (IQR: 1.60-2.90) mmol/L; P = 0.000]. Of them, 10 patients receiving RCS throughout the day for over four weeks had sustained improvement in FBG compared to pre-

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Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

Cited by 17 publications

References 42 publications

Adult focal β-cell nesidioblastosis: A case report

Adult focal β-cell nesidioblastosis: A case report

An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS)

Efficacy of Raw Corn Starch in Insulinoma-Related Hypoglycemia: A Promising Supportive Therapy

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