Epithelioid Hemangioendothelioma

Epithelioid Hemangioendothelioma
Thomas Mentzel, MD
Key Facts
Terminology
  • Vascular neoplasm with metastatic potential composed of epithelioid endothelial cells
Clinical Issues
  • Rare vascular tumor
  • Superficial or deep soft tissue
    • Rare in skin
  • ˜ 50% associated with preexisting vessel
  • Behavior intermediate between hemangioma and angiosarcoma
    • Metastatic rate (20-30%)
    • Mortality (10-20%)
  • Painful mass
  • All age groups
  • Wide local excision with clear margins
  • Adverse prognostic factors
    • > 3 mitoses per 50 high-power fields
    • Tumor size > 3 cm
Macroscopic Features
  • Well-circumscribed nodular lesion
Microscopic Pathology
  • Rare obvious vascular channels
  • Short strands, cords, solid nests, or single cells
  • Bland, epithelioid, round or slightly spindled endothelial cells
  • Intracytoplasmic lumina
  • Myxohyaline, chondroid-like stroma
  • Expression of endothelial markers
    • CD31, CD34, FLI-1
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
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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Epithelioid Hemangioendothelioma

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