Nesidioblastosis Associated with Pancreatic Heterotopia as a Differential Diagnosis of Hypoglycemia: A Literature Review and Case Report

Lopes, Ana Lúcia Miranda; Miranda, Ana Clara; Maior, Marcelo Souto; Mello, Roberto Vieira de; Bandeira, Francisco

doi:10.12659/ajcr.922778

Cited by 9 publications

(10 citation statements)

References 25 publications

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“…Through the detection of proinsulin mRNA and proinsulin in focal nesidioblastosis lesions, it was found that proinsulin mRNA was similar to that in adjacent islets but the level of proinsulin was higher than that in adjacent islets, which indicated that the mutation of these genes effect the level of translation and not transcription [6] . Other metabolic disorders can also result in nesidioblastosis, such as glycogen storage disease, by activating mutations of the monocarboxylate transporter-1, and deficiency of glucose transporter-2 [7] . The pathogenesis of adult nesidioblastosis may be different from infantile nesidioblastosis caused by a genetic effect.…”

Section: Discussionmentioning

confidence: 99%

Adult focal β-cell nesidioblastosis: A case report and literature review

Zhao

2022

Preprint

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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 and literature review

Zhao

2022

Preprint

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“…The acquired form of nesidioblastosis most often occurs in adults following a Roux-en-Y bypass. In this setting, the lesions are more likely to be in the pancreatic tail and surgical resction with distal pancreatectomy has been a treatment for some patients [ 2 , 8 , 27 , 28 ].…”

Section: Discussionmentioning

confidence: 99%

BYPASS-OMA: Hypoglycemic Hyperinsulinemic Nesidioblastosis after Gastric Bypass Surgery—A Case Report and Review of the Literature

Cao

Kim

Huynh

et al. 2022

Case Reports in Endocrinology

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This rare case vignette describes hypoglycemic, hyperinsulinemic nesidioblastosis in a female patient with prior Roux-en-Y gastric bypass. The patient presented with severe symptomatic hypoglycemia resistant to IV dextrose and diazoxide, requiring surgical resection. Traditional imaging found nonspecific findings, and biochemical analysis was inconsistent with insulinoma. A gallium-68 dotatate PET scan was utilized to successfully localize the tumor in the distal pancreas. She underwent laparoscopic resection of the distal pancreatic lesion with resolution of her symptoms and return to euglycemia. The histological evaluation confirmed the diagnosis of nesidioblastosis. Nesidioblastosis is a rare complication of bariatric surgery that may be more clinically relevant with rising prevalence of obesity. Diagnosis with conventional imaging modalities may be challenging; however, the dotatate PET scan may have high utility in detecting lesions. It is essential for clinicians to consider nesidioblastosis in the differential diagnosis of hyperinsulinemic hypoglycemic conditions and recognize there may be a link with increasing rates of bariatric surgery.

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“…Some experts recommend intraoperative ultrasound for localising the functioning tumour directly for pancreatic exploration [ 59 ]. If the pre-operative imaging studies are negative, the intra-arterial calcium stimulation test and assessment of insulin gradient in the gastroduodenal artery is recommended for confirming the regionalisation of insulin excess [ 60 , 61 ]. The diagnostic algorithm suggests verifying the location by performing intraoperative ultrasound and multiple biopsies investigated with histopathologic methods.…”

Section: Surgical Treatmentmentioning

confidence: 99%

“…The diagnostic algorithm suggests verifying the location by performing intraoperative ultrasound and multiple biopsies investigated with histopathologic methods. If the location is not reached, a pancreatic tail resection for further histologic evaluation is recommended [ 60 , 61 ]. Treatment consists of the excision of the focal lesion [ 6 , 39 , 55 ].…”

Section: Surgical Treatmentmentioning

confidence: 99%

Congenital Hyperinsulinaemic Hypoglycaemia—A Review and Case Presentation

Krawczyk

Urbańska

Biel

et al. 2022

JCM

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Hyperinsulinaemic hypoglycaemia (HH) is the most common cause of persistent hypoglycaemia in infants and children with incidence estimated at 1 per 50,000 live births. Congenital hyperinsulinism (CHI) is symptomatic mostly in early infancy and the neonatal period. Symptoms range from ones that are unspecific, such as poor feeding, lethargy, irritability, apnoea and hypothermia, to more serious symptoms, such as seizures and coma. During clinical examination, newborns present cardiomyopathy and hepatomegaly. The diagnosis of CHI is based on plasma glucose levels <54 mg/dL with detectable serum insulin and C-peptide, accompanied by suppressed or low serum ketone bodies and free fatty acids. The gold standard in determining the form of HH is fluorine-18-dihydroxyphenyloalanine PET ((18)F-DOPA PET). The first-line treatment of CHI is diazoxide, although patients with homozygous or compound heterozygous recessive mutations responsible for diffuse forms of CHI remain resistant to this therapy. The second-line drug is the somatostatin analogue octreotide. Other therapeutic options include lanreotide, glucagon, acarbose, sirolimus and everolimus. Surgery is required in cases unresponsive to pharmacological treatment. Focal lesionectomy or near-total pancreatectomy is performed in focal and diffuse forms of CHI, respectively. To prove how difficult the diagnosis and management of CHI is, we present a case of a patient admitted to our hospital.

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Nesidioblastosis Associated with Pancreatic Heterotopia as a Differential Diagnosis of Hypoglycemia: A Literature Review and Case Report

Cited by 9 publications

References 25 publications

Adult focal β-cell nesidioblastosis: A case report and literature review

Adult focal β-cell nesidioblastosis: A case report and literature review

BYPASS-OMA: Hypoglycemic Hyperinsulinemic Nesidioblastosis after Gastric Bypass Surgery—A Case Report and Review of the Literature

Congenital Hyperinsulinaemic Hypoglycaemia—A Review and Case Presentation

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