Abstract
BackgroundHepatic carcinosarcomas, which include both carcinomatous and sarcomatous elements, are uncommon in adults. Although carcinosarcoma in hepatocellular carcinoma is occasionally reported, carcinosarcoma in intrahepatic cholangiocarcinoma (ICC) is an extremely rare ICC variant. Few such cases have been reported in English and no large study of its clinicopathological features exists.Case presentationHere, we report a 60-year-old man with an asymptomatic hepatic B infection who developed hepatic carcinosarcoma from an otherwise normal liver. The 6.0-cm tumor was accidentally discovered by PET-CT in a cancer examination. Serum examinations showed no elevation of tumor markers. He underwent left and caudate lobectomy of the liver. The diagnosis of intrahepatic cholangiocarcinoma with sarcomatous stroma was based on thorough pathologic examination and immunohistochemical staining. The tumor exhibited adenocarcinomatous and sarcomatous components; the adenocarcinomatous element was positive for epithelial markers, the sarcomatous element was positive for mesenchymal markers, but negative for epithelial markers. The patient made an uneventful recovery after surgery. At present, 14 months after surgery, he remains well with no evidence of tumor recurrence.ConclusionsWe report an unusual case of hepatic carcinosarcoma (intrahepatic cholangiocarcinoma with sarcomatous stroma) and discuss the etiology and prognosis of this rare disease.
Highlights
We report an unusual case of hepatic carcinosarcoma and discuss the etiology and prognosis of this rare disease
Hepatic carcinosarcoma (HCS) is a rare tumor, which has been defined by the World Health Organization (WHO) as a malignant tumor containing an intimate mixture of carcinomatous and sarcomatous elements [1]
Few cases have been reported in the English language journals and most have been of HCS in Hepatocellular carcinoma (HCC)
Summary
Primary HCS is very rare worldwide, comprising only 1.8% to 9.4% of surgical or autopsy HCC cases [3, 8]. In 1989, Craig et al [10] first reported liver carcinosarcomas as hepatic tumors with both an HCC and a non-spindle cell sarcoma and excluded non-hepatocytic epithelial elements. In our case, based on IHC findings for our carcinosarcoma specimen, we support the theory that the carcinosarcoma developed from hepatic progenitor cells or stem cells, which differentiates separately into both epithelial and mesenchymal elements. These two different elements were largely separated, a focal area. The results of the present case report supported the etiological theory that sarcomatous elements developed from progenitor or stem cells, rather than redifferentiated from epithelial elements.
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