Epithelioid Hemangioendothelioma



Epithelioid Hemangioendothelioma


Cyril Fisher, MD, DSc, FRCPath










This clinical photograph shows an unusual example of cutaneous epithelioid hemangioendothelioma presenting as a single, discolored, exophytic lesion on the upper back in an adult.






Low-magnification view shows epithelioid hemangioendothelioma with cords of cells image infiltrating the vessel wall and extending into adjacent tissues. The lumen image is filled with thrombotic debris.


TERMINOLOGY


Abbreviations



  • Epithelioid hemangioendothelioma (EHE)


Definitions



  • Angiocentric vascular neoplasm with metastatic potential, composed of epithelioid endothelial cells


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare vascular tumor


  • Age



    • All age groups, but rare in children


  • Gender



    • M = F


Site



  • Skin (rare), superficial or deep soft tissue


  • Extremities, head and neck, viscera (often multicentric)


Presentation



  • Painful mass


  • Solitary mass


  • Multicentric in a number of cases


  • Edema in some cases


  • Occlusion of vessels



    • Due to tumor origin in/association with preexisting vessels


    • Can result in ischemic or venous obstructive symptoms


Treatment



  • Surgical approaches



    • Wide local excision with clear margins


Prognosis



  • Behavior intermediate between hemangioma and angiosarcoma


  • Local recurrence rate 10-15%


  • Metastatic rate 20-30%, mortality 10-20%


  • Superficial cases have better prognosis


  • Adverse prognostic factors



    • > 3 mitoses per 50 high-power fields


    • Tumor size > 3 cm


MACROSCOPIC FEATURES

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Epithelioid Hemangioendothelioma
Premium Wordpress Themes by UFO Themes
%d bloggers like this: