Korean J Med.  2017 Oct;92(5):467-470. 10.3904/kjm.2017.92.5.467.

Malignant Paraganglioma in the Common Hepatic Duct

• Lee HS
• Jeong CU
• Lee ES
• Kwon YJ
• Kim YS
• Kim JD
• Lee YJ

• KMID: 2392333
• DOI: http://doi.org/10.3904/kjm.2017.92.5.467

Abstract

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.

Keyword

Paraganglioma; Hepatic duct, Common; Neoplasms; Metastasis

MeSH Terms

Abdomen
Aged
Ampulla of Vater
Bile Ducts
Dilatation
Hepatic Duct, Common*
Humans
Male
Neoplasm Metastasis
Neural Crest
Paraganglioma*