Teaching Case

A 62-year-old man was admitted to our hospital, sent from peripheral hospital for having large liver mass. Past medical history includes HBV-infection.
Abdominal CT scan showed the presence of a liver mass and pulmonary nodular lesions suggestive of metastasis. Laboratory tests showed elevation of tumour markers AFP and CYFRA 21-1.
The tumour was unresectable and patient died after two months.

An abdominal contrast-enhanced triphasic computed tomography (CT) scan demonstrated a large and predominantly hypodense mass, occupying segments V, VI and VII, with 8 x 6.5 cm, with lobulated contours, displaying progressive peripheral enhancement after intravenous contrast administration, and a central hypodense region suggestive of necrosis.

Sarcomatoid hepatocellular carcinoma (SHC), is an extremely rare form of liver malignant neoplasm, characterised by features of epithelial and mesenchymal tumours. Sarcomatoid carcinoma in general was reported in diverse organs, including lung, breast, urinary tract, skin, head and neck, mucosal region, and small intestine. It may also still be coined as spindle cell carcinoma or carcinosarcoma. [1-2]
SHC is an aggressive variant of hepatocellular carcinoma (HCC) with an incidence of 1.8 - 2% of all surgically resected cases and in 3.9-9.4% of autopsied cases, with either a sarcomatous change in part of the HCC or coexistence of a sarcoma and HCC or cholangiocellular carcinoma (CCC). [3-5]
The precise pathogenesis of sarcomatoid transformation of liver tumours is still unclear, since there are only a few reports of such cases. Clinical studies have suggested that sarcomatoid elements in liver carcinomas result from a dedifferentiation of HCC or CCC. [2-4] Other studies have also suggested that interventional radiological therapies such as transcatheter arterial chemoembolisation (TACE), radiofrequency ablation (RFA), and percutaneous ethanol injection may potentially be a causes of sarcomatoid transformation of hepatocellular carcinoma (HCC). [1-2-3]
Sarcomatoid HCC has a very poor prognosis. Rapid growth, venous invasion, distant and lymph node metastasis and high incidence of recurrence, even after curative resection occur frequently. [2-3]
The diagnosis of SHC is based on immunohistochemical, histopathological and precise pathological techniques. They are positive for cytokeratin-8, desmin, vimentin, SMA, EMA (epithelial membrane antigen) and CD34. Unlike HCC, SHC have low alpha-fetoprotein levels and possess a higher incidence of extrahepatic disease (e.g. lymph nodes, lungs, bone). [1-3]
Surgery is the most effective treatment. Radiofrequency ablation, transarterial chemoembolisation (TACE) and transplantation are other accepted forms of treatment. [1-4]
Written informed patient consent for publication has been obtained.