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

Abstract

BACKGROUNDIntrahepatic 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.AIMTo analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center.METHODSThe 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.RESULTSThe 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.CONCLUSIONIn patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.

Clinical features of sarcomatoid change in patients with intrahepatic cholangiocarcinoma and prognosis after surgical liver resection: A Propensity Score Matching analysis

“…In this study, the median OS following surgical resection for iCCA patients (n = 200) was 16.7 months, similar to that in other reports . After controlling for many confounding factors affecting prognosis through PSM analysis, we reached a consistent conclusion with previous studies that the prognosis of iCCA‐SC is significantly worse than that of ordinary iCCA . For iCCA‐SC, there was a lack of characteristic imaging features from preoperative imaging findings, and there was no significant difference in preoperative serum CEA and CA125 levels between the two groups.…”

“…For iCCA‐SC, there was a lack of characteristic imaging features from preoperative imaging findings, and there was no significant difference in preoperative serum CEA and CA125 levels between the two groups. The serum CA19‐9 levels in the iCCA‐SC group were even lower than those in the ordinary iCCA group, which is in accordance with the study of Kim et al This indicated that these serum tumor markers might not be sensitive for iCCA‐SC, and the diagnosis of iCCA‐SC remained elusive and largely dependent upon pathological findings . Fortunately, this complex situation did not affect the decision of the surgical treatment and the extent of the resection.…”

“…The pathogenesis of iCCA‐SC is still unclear, some researchers have proposed that anticancer treatment might lead to the development of sarcomatoid change or accelerate the transformation of epithelial‐derived cells into sarcoma cells . So far, only a few cases have been reported, most of which have mainly focused on histopathological characteristics . According to previous studies, there is little information on the surgical outcomes and prognostic factors of this malignancy.…”

Clinicopathological characteristics and surgical outcomes of sarcomatoid hepatocellular carcinoma

“…The histogenesis of sarcomatous tissue in cancers, including HCC, has not yet been elucidated. The most widely accepted theory is the conversion theory, which postulates that the sarcomatous element derives from the carcinoma during tumor evolution[ 3 , 9 , 22 - 25 ]. Several recent studies indicate that epithelial-mesenchymal transition is an important mechanism underlying the sarcomatous change, which further confirms the conversion theory[ 26 - 28 ].…”

Sarcomatoid intrahepatic cholangiocarcinoma in a patient with poor prognosis: a case report and literature review

“…According to previous studies, 9,11,12 serum CA19-9 and carcinoembryonic antigen (CEA) may not be sensitive enough for the diagnosis of S-iCCA. Of note, a low echogenic liver mass on ultrasound showing hypoattenuation and peripheral region enhancement after contrast injection on a computed tomography scan 9 are common characteristics of S-iCCA, similar to ordinary iCCA. 7 Therefore, it may be difficult to distinguish S-iCCA from ordinary iCCA using radiological imaging.…”