Skin nonmelanocytic tumors
Vascular tumors
Angiosarcoma

Author: Joel Tjarks, M.D.
Editor: Steven Billings, M.D.

Revised: 15 October 2018, last major update October 2016

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Angiosarcoma [title] skin "loattrfree full text"[sb]

Cite this page: Tjarks, J. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiosarcoma.html. Accessed October 16th, 2018.
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
    • Postradiation (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

Images hosted on other servers:

Scalp, neck and breast lesions

Gross description
  • Violet elevated nodules with ill defined margins
Microscopic (histologic) 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”)
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Hillary Rose Elwood, M.D.

High grade atypical vascular tumor

Atypical vascular tumor involving dermis and subcutis

High power view showing the vascular proliferation dissecting throughout the dermal collagen and lined by atypical endothelial cells



Contributed by Joel Tjarks, M.D.
Missing Image

Various images



Images hosted on other servers:

Secondary angiosarcoma

Positive stains
Negative stains
Molecular / cytogenetics description
  • MYC (8q24) amplification seen in great majority radiation / lymphedema associated tumors
Molecular / cytogenetics images

Images hosted on other servers:

MYC amplification

Differential diagnosis