Analysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years

Abstract

Background: Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is an extremely rare disease, accounting for less than 1% of hepatobiliary system malignancies, and its pathophysiology is not well known. On the hypothesis that its clinical, serologic, or radiologic diagnosis are not fully understood and its prognosis is poor, we investigated the distinguishing features of s-CCC compared with those of intrahepatic bile duct adenocarcinoma [cholangiocellular carcinoma (CCC)] in patients from a single center.

Aim: To analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center.

Methods: The clinical, serologic, imaging, and histopathologic features of 227 patients diagnosed with intrahepatic cholangiocarcinoma (IHCC) in a single medical center during the last 17 years were analyzed. The characteristics of 11 patients with s-CCC were compared with those of 216 patients with CCC.

Results: The number of patients with s-CCC who presented fever and abdominal pain and past history of chronic viral hepatitis or liver cirrhosis (LC) was higher than that of patients with CCC. In imaging studies, patients with s-CCC showed relatively aggressive features. However, no clear distinction was observed between s-CCC and CCC based on other clinical, serologic or radiologic examination results. An accurate diagnosis could be made only via a histopathologic examination through immunohistochemical staining. The clinical course of s-CCC was generally aggressive, and patients had a relatively poor prognosis.

Conclusion: In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.

Keywords: Immunohistochemical staining; Intrahepatic sarcomatoid cholangiocarcinoma; Prognosis; Surgical resection; Survival.

Figure 1

Imaging findings of the patients with various initial diagnoses based on radiologic findings. A: The initial radiologic impressions were intrahepatic cholangiocarcinoma; B: Hepatocellular carcinoma; C: Lymphoma; D: Hepatic abscess, respectively.

Figure 2

Histologic findings of liver biopsy of case 9. A and B: Hematoxylin and eosin stain, × 100; C-E: Immunohistochemistry, × 50; C: Cytokeratin 19; D: Vimentin; E: Hepatocyte specific antigen. The liver shows a mass composed for proliferating anaplastic tumor cells with ovoid or rather spindle shape and no organoid structures (A), and necrosis (B). The tumor cells express CK19 (C) and vimentin (D), not HSA (E).

Figure 3

Comparison of cumulative survival rates. Survival rate of intrahepatic bile duct adenocarcinoma group was better than that of sarcomatoid cholangiocarcinoma group.

Figure 4

Imaging finding of the patient with mixed pathological findings of hepatocellular carcinoma and intrahepatic cholangiocarcinoma. A: Case 1; B: Case 2.