Published online Jan 6, 2023. doi: 10.12998/wjcc.v11.i1.150
Peer-review started: September 1, 2022
First decision: October 24, 2022
Revised: October 27, 2022
Accepted: December 21, 2022
Article in press: December 21, 2022
Published online: January 6, 2023
Processing time: 125 Days and 21 Hours
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.
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.
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.
Core Tip: Focal nesidioblastosis is a neuroendocrine disease which is rarely reported in adults and easily confused with insulinoma, and its pathogenesis is still unclear. We report a rare case of adult focal β-cell nesidioblastosis, which was diagnosed as insulinoma before surgery. Increased glucose level after resection of the lesion may assist in the diagnosis of focal β-cell nesidioblastosis, due to intra-pancreatic regulation in focal nesidioblastosis. We also review the related literature. This case report may enhance clinicians' understanding of this disease.