Cholangiocellular Carcinoma Presenting as Budd-Chiari Syndrome: A Case Report and Literature Review

Toshifumi Wakai, Yoshio Shirai, Jun Sakata, Makoto Inoue, Masahiro Minagawa, Katsuki Muneoka* and Katsuyoshi Hatakeyama

Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences
Department of Surgery, Niitsu Medical Center Hospital*

We herein report the case of a patient with cholangiocellular carcinoma presenting as Budd-Chiari syndrome. A 63-year-old man with shortness of breath and abdominal distension. Thoracoabdominal CT and inferior vena cavography showed inferior vena cava obstruction diagnosed as Budd-Chiari syndrome. Soft tissue density around the obstruction on CT indicating a malignant tumor necessitated exploratory laparotomy, which revealed a 5 cm tumor arising in hepatic segment VIII involving the inferior vena cava. Histologic examination of excisional biopsy specimens showed moderately to poorly differentiated adenocarcinoma. Extrahepatic stromal invasion involved the inferior vena cava and led to Budd-Chiari syndrome. Radical resection was contraindicated due to distant nodal disease and peritoneal metastases. Immunohistochemical study confirmed a diagnosis of cholangiocellular carcinoma, because tumor cells were diffusely positive for cytokeratin 7, a marker for biliary epithelium. He underwent systemic chemotherapy with gemcitabine (250 mg/m²/day) and irinotecan (25 mg/m²/day). No adverse effects were noted. Eight months after chemotherapy was started, the man died of disease. In the literature, we found only 6 cases, including ours, of cholangiocellular carcinoma presenting as Budd-Chiari syndrome. This case and a review of the literature suggest that patients with cholangiocellular carcinoma arising in the liver adjacent to the inferior vena cava may present as Budd-Chiari syndrome and clinicians should consider this in differential diagnosis of Budd-Chiari syndrome.

Key words
cholangiocellular carcinoma, intrahepatic cholangiocarcinoma, Budd-Chiari syndrome

Reprint requests
Toshifumi Wakai Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences
1-757 Asahimachi-dori, Chuo-ku, Niigata, 951-8510 JAPAN

Accepted
December 19, 2007

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