Soft tissue
High grade vascular lesions

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 29 September 2016, last major update November 2012

Copyright: (c) 2003-2016,, Inc.

PubMed search: angiosarcoma [title] soft tissue

Cite this page: Angiosarcoma. website. Accessed October 28th, 2016.
Definition / General
  • Well differentiated (hemangiosarcoma) to anaplastic tumor resembling melanoma or carcinoma
  • Rare; older adults in skin (scalp, face) and soft tissue; also bone, breast, heart, lung, liver, spleen, thyroid (World J Surg Oncol 2012;10:73)
  • Very rare in children and young adults, similar histology but poor prognosis (Am J Surg Pathol 2009;33:264)
  • Arises from endothelial cells of blood vessels; includes aorta, inferior vena cava, pulmonary artery (usually undifferentiated, solid, difficult to identify as endothelial)
  • Risk factors: chronic lymphedema, PVC, radiation, sun exposure, Thorotrast
  • Nodal metastases in 14% (a high rate for sarcomas); also metastases to lungs, liver, bone
  • Breast:
    • 3 - 12 years after radiation therapy for carcinoma
    • Incidence is 1 per 1,000 - 2,000
    • Usually women age 60+ with low grade, low stage lesions
    • Poor prognosis with 3 year survival of 41%
  • Extremities:
    • Associated with lymphedema ~ 10 years after radical mastectomy for breast cancer, arising from dilated lymphatics (lymphangiosarcomas, also called Stewart-Treves syndrome), not associated with radiation therapy
  • Kidney:
    • Unusual neoplasm
    • <15 well documented cases of primary renal angiosarcoma
  • Liver:
    • Associated with arsenic, Thorotrast, PVC
    • Latent period of years
    • Also associated with radiation to other sites, introduction of foreign material
  • Lung:
    • Rarely presents as diffuse pulmonary hemorrhage due to metastases in young adults (Arch Pathol Lab Med 2001;125:1562)
    • Lung metastases often multiple peripheral nodules accompanied by infiltrates, primary tumor usually not identified at presentation; tumor cells may have minimal atypia, may be keratin+; primary site is often heart (Mod Pathol 2001;14:1216)
  • Skin:
    • Cases related to chronic lymphedema are usually in extremities
    • Lymphedema due to radical mastectomy, postfilarial, congenital
    • Cases unrelated to lymphedema are often in head and face
  • Spleen:
    • Rare neoplasm that often remains asymptomatic until massive intra abdominal bleeding
  • Other sites::
    • Small intestine
    • Ovary
Case Reports
  • Early surgery, but survival >5 years is rare
Clinical Images

Images hosted on PathOut server:

Nodular, scalp, courtesy of Dr. Mark R. Wick

Hemangioma like, courtesy of Dr. Mark R. Wick

Cutaneous, courtesy of Dr. Mark R. Wick

Stewart Treves syndrome, courtesy of Dr. Mark R. Wick

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Glistening erythematous ulcerated plaque


Gross Description
  • Early: small, sharply demarcated, asymptomatic, multiple red nodules
  • Late: fleshy, grayish white with hemorrhage, necrosis, deeply invasive
Gross Images
Images hosted on PathOut server:

Deep gluteal muscles, courtesy of Dr. Mark R. Wick

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Micro Description
  • Atypical vascular spaces lined by endothelial cells with cytologic atypia, multilayering
  • In more solid areas are intracytoplasmic lumina containing red blood cells
  • Involves subcutaneous tissue
  • Variable grade (Stanford University)
  • Multinucleated cells may have prominent hyaline globules containing alpha-1-antitrypsin and alpha-1-antichymotrypsin
  • Brisk mitotic activity and necrosis are common
  • Post radiation lesions usually high grade
Micro Images
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Images courtesy of Dr. Mark R. Wick:

Spindle cell type, scalp

Spindle cell type, FLI1 stain


Granular cell type


Epithelioid type, skin

Epithelioid type

Epithelioid type, face

Epithelioid, deep soft tissue

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Lung: metastatic tumor

Lung: metastatic tumor

Site unknown

Skin (image on right is epithelioid)


Thyroid gland

Contributed by Dr. Deepa Santhosh, Banaras Hindu University (India):

Thigh mass

Cytology Images
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Positive Stains
Molecular / Cytogenetics Description
Differential Diagnosis