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

Elżbieta Moszczyńska1, Arnika Wydra2, Klaudia Zasada1

  • 1The Children’s Memorial Health Institute, Department of Endocrinology and Diabetology, Warsaw, Poland

Journal of Clinical Research in Pediatric Endocrinology
|September 1, 2022
PubMed

Insights

Malignant insulinoma, a rare pediatric pancreatic neuroendocrine tumor (PanNET), caused severe hypoglycemia. A successful liver transplant and somatostatin analog therapy led to complete remission in a young patient.

Area of Science:

  • Pediatric Oncology
  • Hepatobiliary Surgery
  • Endocrinology

Background:

  • Insulinoma, a type of pancreatic neuroendocrine tumor (PanNET), is rare in children and causes hypoglycemia due to excess insulin.
  • Malignant insulinoma with liver metastases presents a significant therapeutic challenge, particularly in pediatric cases.

Observation:

  • A 13-year-old girl presented with hyperinsulinism and hypoglycemia.
  • Imaging revealed a pancreatic lesion and liver metastases, necessitating a modified Whipple’s procedure and subsequent allogeneic liver transplantation (LT).

Findings:

  • Histological examination confirmed PanNET. Post-transplant, positron emission tomography-CT using 68Ga-DOTA-labeled somatostatin analogs (SSAs) confirmed complete metabolic remission at age 22.
  • The patient is currently managed with immunosuppressive and anti-proliferative treatments.

Implications:

  • This case highlights the successful multimodal management of pediatric malignant insulinoma with liver metastases.
  • Liver transplantation combined with SSAs and immunosuppressive therapy offers a viable treatment strategy for advanced PanNET in children.

Keywords:
Hypoglycemiachildreninsulinomaliver transplantation

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