A 26 year old woman presented with epigastric and right upper quadrant pain as well as hepatomegaly. A computed tomography revealed a 4 x 5 cm mass in the right hepatic lobe. Laboratory data were unremarkable. A needle biopsy of the hepatic mass was interpreted as pleomorphic sarcoma. A right hepatic lobectomy three months later showed a 6 x 5 x 2.8 cm, well demarcated, gray white, firm mass with necrosis of variable extent.
The microscopic features of the tumor shown in figures 1 and 2. It is composed of fascicles of spindle shaped cells mixed with anaplastic multinucleated giant cells. Myxoid areas and numerous eosinophilic globules were also seen. The tumor cells showed immunoreactivity for vimentin and were negative for cytokeratin, alpha fetoprotein, epithelial membrane antigen, actin and CD34.1
The histologic features and immunohistochemical profile are characteristic of embryonal sarcoma of the liver.
More than half of the patients with this tumor are between 6 and 10 years of age. Cases in adults are rare. The prognosis is poor although in recent years it has improved with combined modality treatment.2
