8991/3 Embryonal sarcoma


Definitions

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
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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
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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
4
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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



5
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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



6
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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



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