Skin - Nonmelanocytic tumors
Vascular tumors
Angiosarcoma

Author: Joel Tjarks, M.D. (see Authors page)

Editor: Steven Billings, M.D.

Revised: 14 November 2016, last major update October 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Angiosarcoma [title] skin

Cite this page: Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiosarcoma.html. Accessed December 4th, 2016.
Definition / General
  • Malignant neoplasm with vascular differentiation
Essential Features
  • Infiltrative vascular neoplasm with broad histologic profile ranging from a well differentiated neoplasm with frank vascular differentiation to a poorly differentiated tumor with epithelioid or spindled cells
  • May mimic poorly differentiated carcinoma, inflammatory process, lymphoma or melanoma
Terminology
  • Also known as hemangiosarcoma
Epidemiology
  • Classically arises in one of three scenarios:
    • Head and neck of the elderly
    • Chronic lymphedema
    • Post radiation (usually in the setting of breast cancer)
Sites
  • Sun exposed skin of the elderly (head and neck); breast with history of lymphedema or radiation therapy
Clinical Features
  • Wide age range (most common in adults)
  • Presents as purple nodules or plaques
  • Highly aggressive
  • Frequent recurrence and metastasis
Prognostic Factors
  • Poor prognosis - high mortality
  • Epithelioid tumors are often more aggressive
Case Reports
Treatment
  • Surgical resection with negative margins
  • Chemotherapy is occasionally used
Clinical Images
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Scalp, neck and breast lesions

Gross Description
  • Violet elevated nodules with ill defined margins
Gross Images
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Hemorrhagic mass

Micro Description
  • Infiltrating, freely anastomosing channels lined by spindled to epithelioid endothelial cells with variable atypia, surrounding adnexae and dissecting dermal collagen
  • Endothelial cells may have multilayered appearance
  • May have free floating intraluminal endothelial cells (“fish in the creek”)
Micro Images

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Missing Image

Contributed by Joel Tjarks, M.D.



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Secondary angiosarcoma

Positive Stains
Negative Stains
Molecular / Cytogenetics Description
  • MYC (8q24) amplification seen in great majority radiation / lymphedema associated tumors
Molecular / Cytogenetics Images
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MYC amplification

Differential Diagnosis