Epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma
Diagnosis in short
Epithelioid hemangioendothelioma. H&E stain.
LM	large epithelioid perivascular cells with abundant pale eosinophilic cytoplasm and cytoplasmic vacuolation ("blister cells") - may form lumen and have RBC within, vesicular nucleus +/-prominent nucleolus; tuft-like projections into capillaries; cells may be in well-circumscribed paucicellular nodules or poorly formed cellular aggregates
LM DDx	epithelioid angiosarcoma, hemangioma
IHC	CD31 +ve, CD34 +ve, factor VIII +ve
Site	soft tissue - see vascular tumours
Prevalence	rare

Epithelioid hemangioendothelioma, abbreviated EHE, is rare malignant vascular tumour.

It should not be confused with epithelioid hemangioma.

General

• Malignant.
• Adults - wide age range.
• Associated with oral contraceptives, vinyl chloride.

Microscopic

Features:

• Large epithelioid perivascular cells with:
  • Abundant pale eosinophilic cytoplasm.
  • Cytoplasmic vacuolation (some cells) - AKA "blister cells" - key feature.
    • May form lumen and have RBC within.
  • Vesicular nucleus with prominent nucleolus in some cells.
• Tuft-like projections into capillaries.
• Tumour cells may be in well-circumscribed paucicellular nodules or more cellular poorly formed aggregates.

DDx:

• Angiosarcoma, epithelioid.
• Hemangioma.

Images

• 

  Epithelioid hemangioendothelioma. (WC)

www:

• Epithelioid hemangioendothelioma - low mag. (flickr.com/Rosen).
• Epithelioid hemangioendothelioma - high mag. (flickr.com/Rosen).
• Epithelioid hemangioendothelioma (surgicalpathologyatlas.com).

IHC

Features:

• CD31 +ve.
• CD34 +ve.
• Factor VIII +ve.

See also

• Vascular tumours.