Huge subcapsular hematoma caused by intrahepatic sarcomatoid cholangiocarcinoma

Intrahepatic sarcomatoid cholangiocarcinomais is a very rare disease with a poor prognosis due to its biologically aggressive tumor behavior. We report a patient who presented with subcapsular hemorrhage and a rapidly growing liver mass. A 57 year-old man was admitted with severe abdominal pain. CT and MRI images showed the presence of a 10 cm-sized subcapsular hemorrhage connected with a multi-lobulated mass with hemorrhage and necrotic foci in the right liver. The patients underwent right hemihepatectomy with caudate lobectomy and lymphadenectomy. The operation findings revealed metastatic nodules to the diaphragm and omentum. Detailed histopathological analysis through immunohistochemistry confirmed the diagnosis of sarcomatoid cholangiocarcinoma with a poorly undifferentiated sarcomatous component. The patient underwent chemotherapy. To date, the patient is doing well for 8 months after initial diagnosis.