Primary hepatic undifferentiated pleomorphic sarcoma co-existing with hepatocellular carcinoma

A 60-year-old man presented with a 1-month history of lacking in strength. He had a history of hepatitis B. Laboratory evaluation revealed the following: alpha fetoprotein, 7.05 ng/mL (normal level, 0-7.00 ng/mL); neuron-specific enolase, 52.20 ng/mL (normal level, 0-20.00 ng/mL). Other tumor markers were normal, including carcinoembryonic antigen and cancer antigen 199. Abdominal MR demonstrated a 1.5 cm diameter nodule and a 7.0 cm diameter mass, both with inhomogeneous hyperintensities on T2WI, fat-suppressed T2WI and diffusion weighted image. After Gd-EOB-DTPA enhancement, the small nodule showed rapid enhancement at arterial phase and washout at portal venous phase, with decrease in Gd-EOB-DTPA uptake at hepatobiliary phase. The big mass showed rim-like enhancement at arterial phase and portal venous phase, with similar decrease in Gd-EOB-DTPA uptake at hepatobiliary phase. Partial hepatectomy was performed and pathological examination of the tissues indicated that the small nodule was highly differentiated hepatocellular carcinoma. However, a gross examination of the big mass revealed grayish white solid tissue. The mass was finally diagnosed as undiferentiated pleomorphic sarcoma, with immunohistochemical results as follows: CKpn (-), Vimentin (+), Glypican3 (-), HepPar-1 (-), CK19 (-), S-100 (-), SMA (-), Desmin (-), MyoD1 (-), Myogenin (-), caldesmon (+), CD117 (-), D0 g-1 (-), CD34 (-), CD99 (+), CD68 (+), CD56 (-), CD21 (-), CD23 (-), EMA (-), S0X-10 (-), Melan-A (+), Ki67 (60%+). The patient was feeling well at 1 month of follow-up.