Soft tissue

Vascular

Intermediate (locally aggressive / rarely metastasizing)

Epithelioid hemangioendothelioma


Editorial Board Member: Borislav A. Alexiev, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Iva Brčić, M.D., Ph.D.
Bernadette Liegl-Atzwanger, M.D.

Topic Completed: 19 January 2021

Minor changes: 21 September 2021

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PubMed search: epithelioid hemangioendothelioma soft tissue pathology review [pt]

Iva Brčić, M.D., Ph.D.
Bernadette Liegl-Atzwanger, M.D.
Page views in 2020: 15,453
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Cite this page: Brčić I, Liegl-Atzwanger B. Epithelioid hemangioendothelioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuehemangioendotheliomaepithelioid.html. Accessed October 15th, 2021.
Definition / general
  • Malignant endothelial neoplasm that most commonly involves soft tissue, bone, lung, skin and liver; can be locally aggressive and has metastatic potential
  • 2 subtypes defined by WWTR1-CAMTA1 or YAP1-TFE3 rearrangement; former expressing CAMTA1 and latter TFE3 (less specific)
Essential features
  • Endothelial neoplasm that most commonly involves soft tissue, bone, lung, skin and liver
  • Locally aggressive tumor with metastatic potential
  • WWTR1-CAMTA1 rearranged tumors: composed of cords or small nests of large endothelial cells with abundant eosinophilic cytoplasm embedded in a myxohyaline stroma; CAMTA1 positive staining
  • YAP1-TFE3 rearranged tumors: composed of solid nests or pseudo alveolar formations of epithelioid cells enmeshed in a fibrous stroma; TFE3 positive staining
ICD coding
  • ICD-O: 9133/3 - epithelioid hemangioendothelioma, malignant
  • ICD-10: D18.0 - hemangioma
  • ICD-11: 2B5Y & XH9GF8 - epithelioid hemangioendothelioma, NOS
Epidemiology
Sites
Pathophysiology
Etiology
  • Unclear
Clinical features
  • Clinical presentation depends on the tumor location, most frequently pain
  • If arising from a vein, occurring symptoms caused by vascular occlusion, such as edema or thrombophlebitis
  • Recurs locally, may metastasize (usually to lymph nodes and lungs) (Diagn Pathol 2014;9:131, Am J Surg Pathol 1997;21:363)
Diagnosis
  • Diagnostic workup includes radiology, histology (biopsy, resection) with immunohistochemistry and molecular analysis
Radiology description
  • CT scan: poorly circumscribed lesion with ground glass appearance
Radiology images

Images hosted on other servers:

Multifocal liver lesions

Prognostic factors
  • 13% recur, 20 - 30% metastasize (lung, lymph nodes), 13% die of disease; for lung, mortality is 65% (Am J Surg Pathol 1997;21:363)
  • High risk (> 3 mitotic figures per 50 high power fields and size > 3 cm) have 5 year disease specific survival of 59% versus 100% for low risk (Am J Surg Pathol 2008;32:924)
Case reports
Treatment
  • Wide local excision
Gross description
Frozen section description
  • Hypercellular tumor composed of groups of epithelioid cells embedded in a myxohyaline stroma
Microscopic (histologic) description
  • WWTR1-CAMTA1 subtype (classic EHE):
    • Cords, strands or small nests of large endothelial cells with abundant eosinophilic cytoplasm embedded in a myxohyaline stroma
    • Tumor cells have vesicular, round to oval, sometimes indented nuclei
    • Some tumor cells have intracytoplasmic, round, clear vacuoles representing small vascular lumina, which may contain erythrocytes
  • YAP-TFE3 subtype:
    • Solid nests or pseudo alveolar arrangement of epithelioid cells enmeshed in a fibrous stroma
    • Tumor cells have abundant, densely eosinophilic cytoplasm and can form vascular spaces
    • Intracytoplasmic vacuoles are rare
  • Usually minimal mitotic activity, atypia or necrosis
  • Up to 10% of cases exhibit frank malignant features of prominent nuclear pleomorphism, increased mitotic activity, solid growth or necrosis; these tumors resemble epithelioid angiosarcoma and have a more aggressive behavior (Am J Surg Pathol 2008;32:924)
Microscopic (histologic) images

Contributed by Iva Brčić, M.D., Ph.D. and Bernadette Liegl-Atzwanger, M.D.
Poorly circumscribed

Poorly circumscribed

Cords and nests

Cords and nests

EHE in the bone

Tumor within bone

Erythrocytes in lumen

Erythrocytes in lumen

Cords in myxohyaline stroma

Cords in myxohyaline stroma

Cellular atypia

Cellular atypia


Mitosis and atypical cells

Mitosis and atypical cells

Nuclear CAMTA1 staining

Nuclear CAMTA1 staining

Diffuse CD31

Diffuse CD31

Diffuse ERG

Diffuse ERG

CAM5.2 focally positive

CAM5.2 focally positive

Cytology description
Positive stains
Negative stains
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:

YAP1-TFE3 fusion

Sample pathology report
  • Right thigh, excision:
    • WWTR1-CAMTA1 rearranged epithelioid hemangioendothelioma (see comment)
    • Comment: Tumor is composed of cords and small nests of large endothelial cells with abundant eosinophilic cytoplasm embedded in a myxohyaline stroma. Immunohistochemically, the tumor cells are positive for ERG, CD31, CAMTA1, focally positive for keratin and are negative for CD34 and TFE3. The morphology and immunoprofile strongly support the diagnosis of WWTR1-CAMTA1 rearranged epithelioid hemangioendothelioma.
Differential diagnosis
Additional references
Board review style question #1

Which of the following is true regarding epithelioid hemangioendothelioma?

  1. Cut surface is white with myxoid areas
  2. It is a benign neoplasm with vascular differentiation
  3. Most of the cases are negative for CAMTA1
  4. Tumor cells stain positive for ERG
  5. Tumor consists of spindle cells embedded in hyalinized stroma
Board review style answer #1
D. Tumor cells stain positive for ERG

Comment Here

Reference: Epithelioid hemangioendothelioma
Board review style question #2

A 30 year old woman presented with a mass of the trunk. Immunohistochemistry shows positive staining with CD31, CD34, ERG and CAMTA1 and focal positivity with keratin. Which of the following is most likely the correct diagnosis?

  1. Epithelioid angiosarcoma
  2. Epithelioid hemangioendothelioma
  3. Epithelioid hemangioma
  4. Malignant melanoma
  5. Myoepithelioma of soft tissue
Board review style answer #2
B. Epithelioid hemangioendothelioma

Comment Here

Reference: Epithelioid hemangioendothelioma
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