Long-term Survival in a Child with Malignant Insulinoma After Liver Transplantation

J Clin Res Pediatr Endocrinol. 2022 Sep 1. doi: 10.4274/jcrpe.galenos.2022.2022-3-5. Online ahead of print.


Insulinoma belongs to pancreatic neuroendocrine tumors and is immensely rare in children. The tumor leads to severe consequences of hypoglycemia caused by excessive insulin release. We report a pediatric patient with malignant insulinoma linked with liver transplantation due to metastases to this organ. A 13-year-old girl presented with symptoms of hypoglycemia due to hyperinsulinism. In computed tomography (CT), a polycyclic lesion in the head of the pancreas and enlarged lymph nodes were revealed. Modified Whipple's operation was performed, and histological examination confirmed pancreatic neuroendocrine tumor. CT of the body showed an enlarged liver with numerous metastases. Allogeneic liver transplantation was carried out successfully. Positron emission tomography-computed tomography (PET/CT) using 68Ga-DOTA-labeled somatostatin analogs at the age of 22 confirmed the complete metabolic remission. The patient currently remains under immunosuppressive and antiproliferative treatment. Multiple surgical interventions, liver transplantation combined with somatostatin analogs, and immunosuppressive medication could be effective in malignant insulinoma.

Keywords: Hypoglycemia; children; insulinoma; liver transplantation.