Intrahepatic sarcomatoid cholangiocarcinoma: A case report of the youngest patient on record and a review of the condition's characteristics

Abstract

As a rare disease, intrahepatic sarcomatoid cholangiocarcinoma (s-CCC) represents less than 1% of malignancies of the hepatobiliary system and its main clinical symptoms include abdominal pain and fever. Results of pathological examinations, despite being the "gold standard", can easily be confused with hepatocellular carcinoma (HCC). This report is about a 32-year-old male patient who was hospitalized due to occupancy of segment V of the liver for three days and had a history of chronic hepatitis B (CHB) over a 20-year span. Magnetic resonance imaging (MRI) showed a 43 mm × 52 mm-sized liver mass in the V segment, with patchy peripheral enhancement during the arterial phase and rapid wash-out during the portal and late phases. A laparoscopic hepatectomy of segment V, along with cholecystectomy, was performed. Histopathological and immunohistochemical examinations indicated a malignant neoplasm that was positive for vimentin and cytokeratin, with these features providing a positive diagnosis for intrahepatic sarcomatoid cholangiocarcinoma. After surgery, an adjuvant therapy of albumin-paclitaxel combined with gemcitabine regimen was given. No recurrence was found six months after the surgery, with follow-up still ongoing. This report aims to improve the awareness, diagnosis, and treatment of s-CCC.

Keywords: case report; hepatobiliary malignancy; immunohistochemical staining; intrahepatic sarcomatoid cholangiocarcinoma; sarcomatoid change.

Figure 1

Ct images of axial sections: (A) A bulky hypodense mass in segment V of the liver indicates the lesion; arterial phase (B) shown enhancement of peripheral areas and slight progressive centripetal enhancement in portal phase (C); three-dimensional reconstruction of the liver mass in segment V (43 mm × 52 mm-sized) (D).

Figure 2

Contrasted MRI images of the liver: (A) an abnormal signal liver mass in segment V and obvious restricted diffusion on DWI; (B) a hypointense signal and a slightly hyperintense one on T1- and T2-weighted scans respectively; (C) patchy peripheral enhancement in the arterial phase, and (D) rapid washout in the portal and late phases.

Figure 3

Histologic findings (hematoxylin and eosin staining, 40 × ): primary high-grade malignant tumor with patchy necrosis and abundant lymphocyte infiltration. Cancer infiltration was not observed in nerves, in vessels or at the margin of hepatectomy.