Paraganglioma is an unusual neoplasm that is embryologically derived from neural crest cells. The most common location of this neoplasm is the adrenal medulla, where these tumors are known as pheochromocytoma. It is extremely rare that paragangliomas occur in the liver. There are only 7 reports of primary hepatic paraganglioma. A 56-year-old man was referred to XX Medical Center. Hypertension was not found. He had suffered from jaundice, headache and weight loss for the 4 previous weeks, but hypertension was not present. The total bilirubin was 7.7 mg/dl and the CA19-9 level was 56.3 U/dl. The tumor was diagnosed as intrahepatic cholangiocarcinoma on the computed tomography image. After biliary drainage via endoscopic nasobiliary drainage, surgical exploration was carried out; right trisectionectomy with caudate lobectomy, portal vein resection and anastomosis were then done. The final pathological diagnosis was primary hepatic malignant paraganglioma of the intrahepatic duct. There has been no evidence of recurrence on the follow up CT images during the 24 month follow up period.
Adrenal Medulla ; Bilirubin ; Cholangiocarcinoma ; Drainage ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Jaundice ; Liver ; Liver Neoplasms ; Middle Aged ; Neural Crest ; Paraganglioma ; Pheochromocytoma ; Portal Vein ; Recurrence ; Weight Loss