Published online May 27, 2024. doi: 10.4240/wjgs.v16.i5.1449
Revised: March 13, 2024
Accepted: April 15, 2024
Published online: May 27, 2024
Processing time: 117 Days and 5.2 Hours
Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is very rare, and the treatment and prognosis are unclear. Herein, we report the case of a middle-aged female with primary large cell NEC (LCNEC) of the common hepatic duct combined with distal cholangiocarcinoma (dCCA). Additionally, after a review of the relevant literature, we summarize and compare mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease.
A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months. Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign. Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels. Imaging examination revealed a 1-cm tumour in the middle and lower segments of the common bile duct. Pancreaticoduodenectomy + lymph node dissection was performed, and hepatic duct tumours were unexpectedly found during surgery. Pathology suggested poorly differentiated LCNEC (approximately 0.5 cm × 0.5 cm × 0.4 cm), Ki-67 (50%), synaptophysin+, and chromogranin A+. dCCA pathology suggested moderately differentiated adenocarcinoma. The patient eventually developed lymph node metastasis in the liver, bone, peritoneum, and abdominal cavity and died 24 months after surgery. Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours.
The prognosis of MiNEN and pure NEC alone is different, and the selection of treatment options needs to be differentiated.
Core Tip: We reported a rare case of primary large cell neuroendocrine carcinoma (NEC) of the hepatic duct combined with distal cholangiocarcinoma (dCCA). The overall survival was 24 months, and the prognosis was relatively good, which may be related to the early stage and low Ki-67 index of NEC and the early stage and moderate differentiation of the coexisting dCCA. To explore genetic causes, gene sequencing was performed on the two types of cancer tissue. A total of 35 gene mutations were detected in NEC tissue, and 7 gene mutations were detected in adenocarcinoma tissue.