Purpose or Case Report: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, of low malignant potential. It is typically seen in adults, but it can also less frequently affect children.

Because of its multisystem involvement, it can present in various ways both clinically and on imaging. It can present with multiple hepatic lesions and/or with multinodular lung disease simulating interstitial lung disease, infectious disease and metastatic disease among others. Multifocal bone involvement is another presentation of EHE.

One of the radiologist's daily practice challenges is that rare diseases can have significant overlap of imaging features with more frequent diagnoses (that sometimes present with atypical features). It is therefore of high relevance to know the classical and unique imaging features and signature of rare entities such as epithelioid hemangioendothelioma, in order to raise clinical suspicion when needed to direct appropriate diagnostic work-up. Our goal is to present a case of EHE, discuss possible clinical presentations and illustrate classical imaging findings. An overview of clinical implications of this diagnosis and of the available treatment options will also be provided.

Combining multisystem affection and characteristic imaging features, especially MRI features of liver lesions, radiologist can help to make early diagnosis of EHE that is rarely seen in children.
Methods & Materials:
Results:
Conclusions: