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.

Primary liver carcinoma with sarcomatous changes: Analysis of 10 cases

“…Several previous studies suggested that the only way of improving prognosis in PLC‐SC is early detection and complete resection of the tumor . Several previous studies discussed the use of systemic chemotherapy for the treatment of recurrent tumors, and reported improvement of prognosis following such therapy . It should be stressed here, however, that not all recurrent tumors include SCs.…”

“…11,12 Several previous studies discussed the use of systemic chemotherapy for the treatment of recurrent tumors, and reported improvement of prognosis following such therapy. 13 It should be stressed here, however, that not all recurrent tumors include SCs. In one case of our study, the resected recurrent tumors did not show any SCs and were diagnosed as ordinary poorly differentiated HCC by histopathological examination.…”

De novo intra‐pancreatic cholangiocarcinoma following liver transplantation for advanced primary sclerosing cholangitis

“…Finally, the pathology of this patient's de novo carcinoma showed spindle cells, indicative of a sarcomatoid neoplasm, an aggressive variant of invasive carcinoma that portends a worse survival 4. While this type of rare neoplasm is more often associated with pancreatic adenocarcinomas, it does occur in hepatocellular carcinomas (3.9–9.4% of reported cases), and even in cholangiocarcinomas (4.5% of reported cases) 5.…”

Stochastic phenotype switching leads to intratumor heterogeneity in human liver cancer

“…A). Such transitional areas have been suggested to be the result of the metaplastic transformation of epithelial cells to mesenchyme‐like cells …”

“…Such transitional areas have been suggested to be the result of the metaplastic transformation of epithelial cells to mesenchyme-like cells. (28,40,41) To further characterize the heterogeneity in different tumor regions, we generated transcriptional profiles by RNA-seq of each component. Comparison of the sequencing profiles showed more similarities in gene expression between sarcomatoid and metastatic than between carcinomatous and sarcomatoid or metastatic components, supporting the notion that metastasis has emerged from the sarcomatoid component ( Fig.…”