8991/3 Embryonal sarcoma


Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22

Undifferentiated (embryonal) sarcoma of the liver (UES; synonym: malignant mesenchymoma of the liver) is a rare malignant hepatic tumour that is composed of undifferentiated mesenchymal cells and that chiefly occurs in older children.

More than 75% of cases of UES have been diagnosed in children aged 6?15 years 1
Click to access Pubmed
Stocker JT (1998)
An approach to handling pediatric liver tumors.
Am J Clin Pathol 109: S67-72

. Few cases occur in adults. It has been estimated that UES ranks as the third most common primary malignant paediatric liver tumour, after hepatoblastoma and hepatocellular carcinoma, representing 9?15% of hepatic tumours in this age group 2
Click to access Pubmed
Stocker JT, Ishak KG (1978)
Undifferentiated (embryonal) sarcoma of the liver: report of 31 cases.
Cancer 42: 336-48

. The incidence in males and females is equal. There is no pre-existing liver disease 3
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010


The prognosis of these tumours has recently improved with combined modality treatment
Click to access Pubmed
Bisogno G, Pilz T, Perilongo G, Ferrari A, Harms D, Ninfo V, Treuner J, Carli M (2002)
Undifferentiated sarcoma of the liver in childhood: a curable disease.
Cancer 94: 252-7

Click to access Pubmed
Kim DY, Kim KH, Jung SE, Lee SC, Park KW, Kim WK (2002)
Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy.
J Pediatr Surg 37: 1419-23

Click to access Pubmed
Pachera S, Nishio H, Takahashi Y, Yokoyama Y, Oda K, Ebata T, Igami T, Nagino M (2008)
Undifferentiated embryonal sarcoma of the liver: case report and literature survey.
J Hepatobiliary Pancreat Surg 15: 536-44