Abstract
Undifferentiated embryonal sarcoma of the liver is a rare primary mesenchymal hepatic tumor that usually occurs in pediatric patients. In adulthood, this aggressive neoplasm represents only 7% of the liver sarcomas. This case reports a liver sarcoma occurring in a 49-year-old female patient. The patient was admitted in the emergency room with abdominal pain. Computerized tomography scan and magnetic resonance imaging showed a giant lobulated cystic mass in the right hepatic lobe, suggesting an atypical hemangioma. Right hepatectomy was performed. This rare case promotes a review of the differential diagnosis of liver primary neoplasms including sarcoma. The histological examination revealed an undifferentiated embryonal sarcoma. The patient underwent adjuvant chemotherapy. Currently, our patient is in complete sustained remission 4 years after chemotherapy.