Primary Hepatic Neuroendocrine Carcinoma with Multiple Liver Metastases Presenting as Jaundice Due to CBD Obstruction

Abstract

Background: Whereas more than 80% of NETs found in the liver are metastatic, primary hepatic neuroendocrine tumors (PHNETs) are very rare. We experienced a case of primary hepatic neuroendocrine carcinoma (PHNEC) with multiple liver metastases presenting as jaundice due to the mid CBD obstruction. To the best of our knowledge, few studies have reported similar cases like ours in the English literature. Case presentation: A 81-year-old man was admitted with jaundice of two week's duration. His initial laboratory findings were total bilirubin 19.75 mg/dl, direct bilirubin 15.72 mg/dl, aspartate aminotransferase 65 IU/L, alanine aminotransferase 71 IU/L, alkaline phosphatase 525 U/L, gamma-glutamic transpeptidase 313 U/L and CA19-9 529 U/ml. Abdominal CT revealed marked dilatation of intrahepatic bile duct (IHD) and proximal CBD due to CBD invasion by conglomerated lymphadenopathies in the hepatoduodenal ligament and showed subtly enhancing lesion with central hypodense portion in the liver segment 8/5. MRI with MRCP showed multiple variable sized T2 high signal intensity masses (the largest 3.6 cm) in the both hemiliver, which showed diffusion restriction and delayed enhancement, and diffuse IHD and proximal CBD dilatation with conglomerated lymph nodes at portocaval space and hepatoduodenal ligament. Percutaneous transhepatic biliary drainage was performed, but biopsy could not be done because of concomitant hemobilia. So, we performed sono-guided liver biopsy at the intrahepatic masses. Histopathology revealed poorly differentiated carcinoma. The immunohistochemical staining for CD56, synaptophysin and thyroid transcription factor-1 (TTF-1) revealed positive reaction. So, the diagnosis of neuroendocrine carcinoma, large cell type was confirmed. Chest CT for excluding metastasis from extrahepatic primary site or distant metastatic involvement showed no mass-like lesion in the both lung fields. He refused any methods of treatment such as chemotherapy or transhepatic arterial chemoembolization, and wanted only conservative treatment. Therefore, he was discharged after insertion of self-expandable metal stent at the mid CBD obstruction site and he was died 5 months later. Conclusion: Our case suggests that PHNEC can be presented as multiple intrahepatic masses with CBD obstruction even though PHNETs are very rare. Therefore, it is important to confirm the pathology even in such cases.Figure 1Figure 2