Soft tissue

Topic Completed: 1 November 2012

Minor changes: 15 February 2021

Copyright: 2002-2021,, Inc.

PubMed search: Angiosarcoma [title] soft tissue

Vijay Shankar, M.D.
Page views in 2020: 24,038
Page views in 2021 to date: 4,580
Cite this page: Shankar V. Angiosarcoma. website. Accessed February 27th, 2021.
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 intraabdominal bleeding
  • Other sites:
    • Small intestine
    • Ovary
Case reports
  • Early surgery but survival > 5 years is rare
Clinical images

Contributed by Mark R. Wick, M.D.

Nodular, scalp



Stewart-Treves syndrome

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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

Contributed by Mark R. Wick, M.D.

Deep gluteal muscles

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Microscopic (histologic) 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
  • Postradiation lesions usually high grade
Microscopic (histologic) images

Scroll to see all images:

Contributed by Mark R. Wick, M.D.

Spindle cell type, scalp

Spindle cell type, FLI1 stain


Granular cell type


Epithelioid type, skin

Epithelioid type

Epithelioid type, face

Epithelioid, deep soft tissue

Contributed by Dr. Deepa Santhosh

Thigh mass

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Skin (image on right is epithelioid)


Thyroid gland

Positive stains
Molecular / cytogenetics description
Differential diagnosis
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