Proinsulin-Secreting Neuroendocrine Tumors of the Pancreas: A Single-Centre Experience

Background: Proinsulinoma is a neuroendocrine tumor (NET) of the pancreas that produces prohormone proinsulin. These tumors are very rare. In the literature, they are most often presented in the form of case reports. Materials and Methods: We studied 177 patients with NET of the pancreas who underwent surgical treatment in the A.V. Vishnevsky National Medical Research Centre of Surgery from January 2007 to December 2018. Results: Of 81 patients with organic hyperinsulinism caused by functioning NETs of the pancreas during the study period, 3 (3.7%) had a proinsulinoma; 2 were female; and 1 was male. None of them admitted to weight gain during this period, and their BMI was normal. All patients presented with Whipple's triad during the 72-h fast. Tumor-enucleating surgery was performed: one robot assisted, two laparotomies. A normal glucose level after treatment was achieved in all cases. Conclusion: In cases where clinical hypoglycemia is present, but the serum insulin level is within the normal range or even decreased, proinsulinoma should be suspected. For now, surgical resection remains the only effective method of treatment. Further investigation of proinsulinomas is needed.