Primary Hepatic Undifferentiated Pleomorphic Sarcoma: CT and angiographic findings in two cases

Primary hepatic undifferentiated pleomorphic sarcoma (UPS) is an extremely rare disease entity whose imaging characteristics are not well documented in literature. We present two cases of primary hepatic UPS with contrasting disease courses, and their CT and angiographic findings in this report. Imaging characteristics include inhomogeneous appearance and peripheral enhancement pattern, with necrosis and delayed gradual fill-in enhancement more evident in the larger tumor. Although imaging findings are not specific, being familiar with the common manifestations of primary hepatic UPS will allow radiologists to include it in their differential diagnosis list for suspected sarcomatous or mesenchymal tumors of liver. Correspondence Author to: Chi-Jen Chen Department of Radiology, Shuang Ho Hospital, Taipei Medical University, New Taipei, Taiwan No. 291, Zhong-Zheng Road, Zhong-He, New Taipei 23561, Taiwan J Radiol Sci 2013; 38: 15-19 Primary hepatic undifferentiated pleomorphic sarcoma 16 J Radiol Sci March 2013 Vol.38 No.1 Figure 1. 72-year-old man with primary hepatic undifferentiated pleomorphic sarcoma. Pre-contrast a. and post-contrast CT at arterial b., portal venous c. and equilibrium d. phases showed a solitary small hypodense nodule (arrow) with illdefined margin in segment 6 of liver, with a peripheral enhancement pattern. Diagnostic angiography (e. and f.) showed an irregular hypervascular stain (arrow) in right hepatic lobe. Figure 1 Primary hepatic undifferentiated pleomorphic sarcoma 17 J Radiol Sci March 2013 Vol.38 No.1 Figure 2. 64-year-old man with primary hepatic undifferentiated pleomorphic sarcoma. Pre-contrast a. and post-contrast CT at arterial b., portal venous c. and equilibrium d. phases showed a lobulated hypodense mass (arrow) occupying segments 4, 5 and 8 of liver, with an initial peripheral and gradual fill-in enhancement pattern, and large central areas of necrosis. The tumor had ruptured at its lateral margin, with blood clot in right perihepatic space. Diagnostic angiography (e. and f.) showed a huge hypervascular stain with peripheral pattern of enhancement in right side of liver. Figure 2 Primary hepatic undifferentiated pleomorphic sarcoma 18 J Radiol Sci March 2013 Vol.38 No.1 hypervascular stain with peripheral pattern of enhancement in right side of liver, without evidence of active bleeding (Fig. 2e, 2f). Both the reporting radiologist and surgeon were under the impression of a ruptured HCC. He received right lobectomy, followed by systemic chemotherapy after histopathology revealed UPS. Tumors recurred in liver within two months along with multiple lung metastases. He died of fulminant disease at two months after surgery.