WHAT IS HE?

Hemangioendothelioma (HE) is the term used to describe a diverse group of vascular cancers. It can be broken down as follows:

HEM = Blood

ANGIO = Blood vessel

ENDO = Inside

THELIA = Cellular layer or surface

ENDOTHELIUM = Interior lining of a (blood) vessel

OMA = Tumor

The term is descriptive, indicating that the predominant proliferating cell is related to an endothelial cell (the cell that lines the inside of blood vessels).

ehe specifically

Refers to a variety where the cells take on an 'epithelial' appearance. However, it is distinct, both histologically and pathologically and in its cause from the common hemangiomas of infancy. It is generally considered a low grade malignancy based on its histological features.

HE TUMORS

can invole soft tissue, bone, skin, liver, lymph node and/or lung, but there are clear patterns of clinical presentation. For example EHE is most common in the liver, commonly occurs at multiple sites at the same time (metachronous growth), and does not cause coagulopathy.

Kaposiform Hemangioendothelioma (KHE)

typically occurs on the trunk, remains very local and is commonly associated with coagulopathy. In contrast, pattern of progression of these tumors varies greatly and is distinctly different from its benign counterpart, the hemangioma. While in rare cases spontaneous regressions have been reported, these tumors are generally slow and indolent. However, in some cases it can progress to devastating disease. The tumors are often locally invasive and destroy surrounding tissue.

vascular cancers

Ie, cancers pertaining to vessels, particularly blood vessels, are very rare, accounting for only a fraction of 1% of all cancers. Due to it's rarity, it is crucial for patients to seek a sarcoma specialist for the treatment of any hemangioendothelioma.