9133/1 Epithelioid hemangioendothelioma, NOS


Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

A low grade malignant blood vessel neoplasm characterized by the presence of epithelioid endothelial cells, hyalinized and myxoid stroma formation, and angiocentricity. It may present as a painful mass.



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

A tumour of variable malignant potential that is composed of spindle or epithelioid endothelial cells growing along preformed vessels or forming new vessels (angiocentricity) and supported by hyalinized and myxoid stroma.

Epithelioid haemangioendothelioma (EHE) of the liver acquired its name because of the involvement of a neoplastic cell with an epithelioid morphology, but the neoplasm is clearly not epithelial. It is an uncommon tumour that presents between the ages of 12 and 86 years. The overall incidence of this tumour is unknown, but more hepatic EHEs are reported in females (about 60%) than in males (about 40%), in contrast to EHEs arising in soft tissues. Most patients are adults; of 137 patients with EHE of the liver, only seven patients were aged 20 years or < 20 years 1
Click to access Pubmed
Makhlouf HR, Ishak KG, Goodman ZD (1999)
Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases.
Cancer 85: 562-82



. It may present as a painful mass. It is generally behaves as a low-grade malignant neoplasm with slow progression, but appears to be resistant to chemotherapy and may undergo a fatal course 2
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Uchimura K, Nakamuta M, Osoegawa M, Takeaki S, Nishi H, Iwamoto H, Enjoji M, Nawata H (2001)
Hepatic epithelioid hemangioendothelioma.
J Clin Gastroenterol 32: 431-4



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



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Bronchus and lung
ICD-O-3 topography code: C34

A low grade malignant blood vessel neoplasm characterized by the presence of epithelioid endothelial cells, hyalinized and myxoid stroma formation, and angiocentricity. It may present as a painful mass.