Soft tissue
High grade vascular lesions
Angiosarcoma

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

Revised: 29 September 2016, last major update November 2012

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

PubMed search: angiosarcoma [title] soft tissue

Cite this page: Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueangiosarcoma.html. Accessed December 7th, 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
Sites
  • 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
Treatment
  • 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



Images hosted on other servers:

Foot

Glistening erythematous ulcerated plaque

Nose

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



Images hosted on other servers:

Ovary

Spleen

Thyroid

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 hosted on other servers:

Images courtesy of Dr. Mark R. Wick:

Spindle cell type, scalp

Spindle cell type, FLI1 stain

Scalp

Granular cell type

Face

Epithelioid type, skin


Epithelioid type

Epithelioid type, face

Epithelioid, deep soft tissue



Images hosted on other servers:

Breast

Ovary

Nose


Lung: metastatic tumor

Lung: metastatic tumor

Site unknown


Skin (image on right is epithelioid)

Spleen

Thyroid gland



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

Thigh mass

Cytology Images
Images hosted on other servers:

Kidney

Positive Stains
Molecular / Cytogenetics Description
Differential Diagnosis