Epithelioid Hemangioendothelioma



Epithelioid Hemangioendothelioma


Thomas Mentzel, MD









Clinical photograph shows a rare cutaneous epithelioid hemangioendothelioma presenting as an exophytic lesion.






Hematoxylin & eosin shows an epithelioid hemangioendothelioma arising in deep soft tissue as an ill-defined angiocentric neoplasm.


TERMINOLOGY


Abbreviations



  • Epithelioid hemangioendothelioma (EHE)


Synonyms



  • Intravascular bronchioloalveolar tumor


  • Angioglomoid tumor


Definitions



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


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare vascular tumor


  • Age



    • All age groups


    • Rare in childhood


  • Gender



    • M = F


Site



  • Superficial or deep soft tissue


  • Extremities


  • Head & neck region


  • Rare in skin


  • Visceral organs (often multicentric)


Presentation



  • Painful mass


  • Solitary mass


  • Multicentric in a number of cases


  • Edema



    • May be present


  • Occlusion of vessels



    • 1/2 of cases arise in/are associated with preexisting vessels


    • May cause more profound symptoms


Treatment



  • Surgical approaches



    • Wide local excision with clear margins


  • Adjuvant therapy



    • No adjuvant chemo-/radiotherapy


Prognosis



  • Intermediate behavior between hemangioma and angiosarcoma


  • Local recurrence rate (10-15%)


  • Metastatic rate (20-30%)


  • Mortality (10-20%)


  • Better prognosis in superficial cases


  • Adverse prognostic factors



    • > 3 mitoses per 50 high-power fields


    • Tumor size > 3 cm


IMAGE FINDINGS


General Features



  • Best diagnostic clue



    • May show calcifications


  • Location



    • Soft tissue of extremities


MACROSCOPIC FEATURES


General Features



  • Well-circumscribed nodular lesion


  • Fusiform intravascular mass resembling organizing thrombus


MICROSCOPIC PATHOLOGY


Histologic Features



  • Expansion of vessels in angiocentric cases


  • Centrifugal extension into soft tissues




  • Rare obvious vascular channels


  • Short strands, cords, solid nests, single cells


  • Round to slightly spindled endothelial tumor cells


  • Eosinophilic cytoplasm


  • Vesicular nuclei


  • Small nucleoli


  • Intracytoplasmic vacuoles



    • Represent miniature endothelial lumina


    • May contain erythrocytes


  • Bland epithelioid tumor cells


  • Rare mitoses


  • Myxohyaline stroma


  • Chondroid stroma


  • Metaplastic calcification &/or ossification in ˜ 10% of cases


  • Stroma contains sulfated acid mucopolysaccharides


  • Atypical features in ˜ 1/3 of cases



    • Increased cellularity


    • Solid nests


    • Marked nuclear atypia


    • Enlarged nuclei


    • Prominent nucleoli


    • Mitoses


    • Spindling of tumor cells


    • Necrosis

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Epithelioid Hemangioendothelioma

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