Published online Feb 7, 2019. doi: 10.3748/wjg.v25.i5.608
Peer-review started: October 31, 2018
First decision: December 20, 2018
Revised: January 3, 2019
Accepted: January 14, 2019
Article in press: January 14, 2019
Published online: February 7, 2019
Processing time: 91 Days and 6.4 Hours
Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is extremely rare, and its pathophysiology is not well known. Because of the poor prognosis, early diagnosis and aggressive treatment is important.
Clinical, serologic, and imaging findings are known to be not helpful to distinguish s-CCC from intrahepatic bile duct adenocarcinoma (CCC) or other hepatic tumors. There is no established treatment option to prolong survival, except surgery.
This study aimed to analyze the distinct characteristics of s-CCC patients for early diagnosis and appropriate treatment.
This retrospective study was conducted in a single center of South Korea for assessment of 11 patients with s-CCC diagnosed for 17 years. We analyzed the clinical, serologic, imaging, and histopathologic features of s-CCC patients and compared with those of CCC patients.
The patients with s-CCC tended to present abdominal pain or fever as the chief complaint and have past history of liver cirrhosis (LC) or chronic viral hepatitis more frequently, compared with the patients with CCC. In addition, s-CCC showed relatively aggressive features on imaging studies. However, no clear distinction in other clinical and serologic, or radiologic examination results between s-CCC and CCC patients. Only a histopathologic examination with immunohistochemical staining was helpful and essential for an accurate differential diagnosis of s-CCC. The clinical course of s-CCC was relatively aggressive, and patients had poor prognoses. Surgery is generally recommended first, however in this study, we could not obtain meaningful results of the surgical treatment or chemotherapy for s-CCC.
s-CCC is extremely rare disease which presents aggressive clinical course and poor prognosis. Clinical, serologic, and imaging studies are not helpful in diagnosis of s-CCC. In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.
Although s-CCC has a poor prognosis, its pathogenesis and the effects of non-surgical treatment are not well established. In addition, there is no definite strategy for differential diagnosis except a histopathological examination. A large-scale, prospective, and multicenter study involving larger number of patients should be conducted in the future.