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The article reports that F-Dihydroxyphenylalanine positron emission tomography is the preferred method for differentiation between focal and diffuse congenital hyperinsulinism and focus localization. Surgeons manages to perform curative limited resection of a focus without the risk of long-term diabetes through the sensitivity and accuracy in preoperative focus localization.

Introduction: Insulinomas are extremely rare tumors in children and an uncommon first manifestation of MEN1 syndrome. Insulinomas are usually benign tumors with only a few reports of malignant cases in children. Aim(s): To investigate the morphological and genetic characteristics of Russian children with primary insulinomas. Materials and methods: Insulinoma was diagnosed biochemically and by imaging and was verified histopathologically. Sequencing of the MEN1 gene was performed and families of the mutation carriers were studied. Follow-up included screening for signs of MEN1 and metastases in case of malignant tumors. Results: Ten children aged 8-16 years were diagnosed to have primary pancreatic insulinoma. Of these, five (50%) were malignant and distant metastases were seen during follow-up in two children. Three out of nine children investigated (33.3%) were found to have mutations in the MEN1 gene and developed hyperparathyroidism and hyperprolactinemia during the following 10 years. One patient with w/t MEN1 developed hyperparathyroidism. In the two patients with MEN1 mutations where family biochemical testing was possible, relatives revealed the spectrum of MEN1 components, but with no clinical symptoms. Conclusion: MEN1 syndrome should be suspected in all cases of pediatric insulinomas. A high incidence of malignant insulinomas was seen. Contrary to the ENETS Guidelines, we recommend MEN1 analysis, histopathological investigations and specific follow-up in all children with primary insulinoma, and family testing in case of MEN1 mutations. [ABSTRACT FROM AUTHOR]