Imaging and clinical features of primary hepatic sarcomatous carcinoma

BACKGROUND: Primary hepatic sarcomatous carcinoma (PHSC) is a rare malignancy composed of both carcinomatous (either hepatocellular or cholangiocellular) and sarcomatous components. The purpose of our study was to evaluate the imaging and clinical findings of PHSCs, improving the understanding and diagnosis of tumors. METHODS: We retrospectively reviewed the imaging and clinical findings of ten patients with pathologically proven PHSCs, including two cases of sarcomatous intrahepatic cholangiocarcinoma (S-ICC), seven cases of sarcomatous hepatocellular carcinoma (S-HCC) and one case of sarcomatous combined hepatocellular and cholangiocarcinoma (S-HCC–CC). Six patients underwent computed tomography (CT) scans and five underwent magnetic resonance imaging (MRI) scans with one of them having both CT and MRI scans. RESULTS: Eight of ten patients had a background of chronic hepatitis or cirrhosis. The elevation of alpha-fetoprotein (AFP) was positive in half of the patients. All the tumors were located near the liver subcapsular area and six of ten cases were massive with round or oval shapes and ill-defined. The lesion textures were mainly heterogeneous in eight tumors for the necrosis or hemorrhage. Eight tumors showed hypo-enhancement and nine tumors exhibited initial peripheral rim (five cases) or heterogeneous (four cases) enhancement, followed by progressive (six cases) and peripheral or partial washout (three cases) on the later phases. Of the seven surgically resected tumors, five showed liver capsular invasion with one of them rupturing into the perihepatic space. Vascular thrombosis (five cases), intrahepatic metastasis (four cases), adjacent organ invasion or seeding (three cases), and lymph node metastasis (four cases) were found on imaging or in pathology. The follow-up period ranged from one to 36 months. Four patients with T3-T4 staging died from recurrence and metastasis between 2 and 5 months, and three patients with T1 staging did not have any recurrence between 16 and 24 months. CONCLUSION: PHSC generally presents as a subcapsular mass with hypovascularity and may be characterized by rim-like or heterogeneous enhancement on the arterial phase and a progressive dynamic pattern. These tumors usually coincide with chronic hepatitis or cirrhosis and poor prognosis appears to be associated with TNM staging.