Intrahepatic sarcomatoid cholangiocarcinoma

Abstract

Intrahepatic sarcomatoid cholangiocarcinoma is a rare but an aggressive variant of cholangiocarcinoma with a very poor prognosis. We report the first caucasian patient who presented with a rapidly enlarging liver mass requiring hepatic resection. Detailed histopathological analyses including immunohistochemistry and electron microscopy confirmed sarcomatoid cholangiocarcinoma. The patient had early onset disease recurrence within 5 weeks of surgery. Here we demonstrate that combination chemotherapy with gemcitabine and cisplatin is a potential treatment option in patients with advanced sarcomatous cholangiocarcinoma. The patient achieved sustained partial remission with combination chemotherapy and remains alive and well more than 29 months since initial surgery.

Figure 1

Radiological and histopathological features are shown here. Figure 1(a): preoperative CT-scan, Figure 1(b): gross histopathology and Figure 1(c): microscopy features. See text for details.

Figure 2

Postoperative CT-scan showing early disease recurrence (indicated with an arrow) within the liver (Figure 2(a)). Partial response after completion of chemotherapy is shown in Figure 2(b).