Carcinoid syndrome.

Area of Science:

• Pediatric Oncology
• Surgical Gastroenterology
• Hepatology

Background:

• Carcinoid syndrome (CS) is rare in children, often arising from ileal neuroendocrine tumors with liver metastasis.
• Standard treatments like surgery, octreotide, and chemotherapy showed limited efficacy in this pediatric CS case.
• Liver transplantation is generally not favored for metastatic liver disease but considered for slow-growing neuroendocrine tumors.

Purpose of the Study:

• To report a rare case of pediatric carcinoid syndrome successfully managed with liver transplantation.
• To evaluate the efficacy of liver transplantation in a pediatric patient with metastatic neuroendocrine tumor causing CS.
• To highlight the potential of liver transplantation in managing refractory pediatric carcinoid syndrome.

Main Methods:

• Resection of the primary ileal carcinoid tumor.
• Administration of octreotide and polychemotherapy (5-fluorouracil, epidoxorubicin, deticene).
• Performance of a liver transplantation due to treatment failure and poor prognosis.

Main Results:

• Initial treatments failed to control metastatic liver disease and clinical symptoms of CS.
• The child achieved complete clinical and laboratory remission 4 months post-liver transplantation.
• This case adds to the limited reported instances of CS in pediatric patients.

Conclusions:

• Liver transplantation can be a viable and effective treatment option for pediatric carcinoid syndrome with extensive liver metastases.
• Neuroendocrine tumors' slow-growing nature may contribute to better outcomes with liver transplantation compared to other metastatic liver diseases.
• This case underscores the importance of considering advanced surgical options for rare pediatric malignancies when standard therapies are insufficient.