脳障害と治療

概要

Primary Neuroendocrine Tumor of the Portal Hepatic Duct in Adolescence: A Case Report

Fanghong Wang, Kexiang Zhu, Lei Zhang, Hui Zhang, Bo Zhang, Fanghui Ding, Xun Li*

Background: It is not uncommon for Neuroendocrine Tumors (NETs) to occur in the gastrointestinal tract, pancreas, and lungs, but NETs originating from the biliary system are relatively rare, accounting for 0.67% of gastroenteropancreatic system tumors. At present, less than 10 cases of extrahepatic biliary neuroendocrine tumors have been reported in adolescent.

Case presentation: This report presents a case of a 16-year-old boy with a NET of the portal hepatic duct. Obstructive jaundice was judged from the laboratory examination and imaging findings. After the evaluation and multidisciplinary discussion, he underwent resection of the common bile duct, common hepatic duct, and gallbladder and Roux-en-Y hepaticojejunostomy. Pathological examination revealed a NET with the tumor invading the wall of the bile duct and surrounding adipose tissue. The patient had no NETs in any other organs or tissues. This case was a primary NET, not a metastatic tumor.

Conclusion: The preoperative diagnosis of primary neuroendocrine tumor of the portal hepatic duct is difficult, and this tumor is often confused with hilar cholangiocarcinoma. Surgical treatment is the only effective way to cure it. Once the diagnosis has been made, surgical resection should be performed as soon as possible.