Comparison Between Sporadic and Multiple Endocrine Neoplasia Type 1-Associated Insulinoma.

BACKGROUND: The differences between sporadic and MEN-1 associated insulinoma are not well described. Herein, we compared demographics, neoplasm characteristics, presentation, and survival in patients with sporadic vs MEN-1 insulinomas including benign and malignant disease.

METHODS: A retrospective study identified insulinoma patients. MEN-1 was defined based on genetic testing or clinically in patients with two or more primary MEN-1 tumor types.

RESULTS: A total of 311 patients were identified: 84% benign and 16% malignant. The incidence of malignancy was similar (18% vs. 16%, MEN-1 vs. Sporadic, p = 0.76). Within malignant patients, the median (IQR) age was 33 (25,44) in MEN-1 vs 54 (41,70) in sporadic insulinoma, p=0.04. There was no difference in sex or tumor size between MEN-1 and sporadic malignant insulinoma, p>0.05Of the 260 patients with benign insulinoma, 7% had MEN-1 syndrome. MEN-1 patients presented with insulinoma at a younger age: median (IQR) age was 38 (24, 49) vs 52 (43, 65) years, p<0.01.Resection of benign insulinoma was performed in 78% of the MEN-1 and 94% of the sporadic group, p=0.03. Resected benign tumors were larger in the MEN-1: 2 (1.65, 2.45) cm vs 1.5 (1.2, 2.0) cm, respectively, p=0.03. Concurrent insulinomas were more common in MEN-1 (17% vs 2%), p <0.01.

CONCLUSION: MEN-1 patients present with insulinoma at younger age and have larger benign pancreatic lesions at the time of resection compared to sporadic neoplasms. Younger patients and those with multifocal pancreatic neuroendocrine tumor in the setting on endogenous hyperinsulinism should be evaluated for MEN-1.