RESUMEN
Paragangliomas are rare extra-adrenal neoplasms of neural crest origin. The neoplasms may develop at various sites, but most are located in the para-aortic space along the sympathetic chain. A paraganglioma in the bile duct is very rare; only four cases of such tumors in the hepatic bile duct have been reported to date. Herein, we report on the first Korean case of a malignant paraganglioma in the common hepatic duct (with hepatic metastases) in a 75-year-old male. Computed tomography of the abdomen revealed a heterogeneously enhancing lesion in the common hepatic duct with dilatation of the intrahepatic ducts. After balloon sweeping, the mass exited spontaneously through the Ampulla of Vater. The mass was about 1.5 × 1.3 × 0.5 cm in its dimensions and the surface appeared to be necrotic and edematous. Microscopically, the tumor cells were arranged in a Zellballen pattern. The tumor was diagnosed as a malignant paraganglioma.