Ruptured Undifferentiated (Embryonal) Sarcoma of the Liver in an Adult

ABSTRACT

The undifferentiated (embryonal) sarcoma of the liver (USL) has previously been called malignant mesenchymoma, undifferentiated sarcoma and fibromyxosarcoma. USL was named as an entity by Stocker and Ishak in 1978 on the basis of an Armed Forces Institute of Pathology (AFIP) series of 31 cases. The USL is a rare primary neoplasm of a mesenchymal origin and it predominantly occurs in children. Stocker reported that it was fourth in frequency among the liver tumors of childhood, following hepatoblastoma, hemangioendothelioma and hepatocellular carcinoma. Although there has been controversy as to the most appropriate treatment, the studies have reported that long term survival is possible after complete surgical resection with or without perioperative chemotherapy. This tumor's frequency in the adult population is extremely low. We report here on a case of USL in an adult woman with the review of the relevant literature.