Soft Tissue
High grade vascular lesions
Angiosarcoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)

Revised: 15 October 2014, last major update November 2012

Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

PubMed Search: Angiosarcoma [title] soft tissue

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General
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• 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
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• 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
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• 22 year old woman with angiosarcoma in recurrent malignant phyllodes tumor of breast (Indian J Pathol Microbiol 2012;55:264)
• 25 year old woman with small intestine tumor (Ann Afr Med 2011;10:246)
• 29 year old woman with bilateral breast lumps (Indian J Med Paediatr Oncol 2011;32:160)
• 35 year old woman with cutaneous angiosarcoma complicating morbid obesity (Arch Pathol Lab Med 2001;125:531)
• 50 year old man with kidney mass (Arch Pathol Lab Med 2002;126:478)
• 60 year old man with splenomegaly and fever (Indian J Med Paediatr Oncol 2011;32:230)
• 69 year old man with raised lesion on nose (Indian J Dermatol Venereol Leprol 2012;78:496)
• 80 year old woman with epithelioid angiosarcoma (Indian J Dermatol Venereol Leprol 2012;78:494)
• 81 year old woman with ovarian tumor (Diagn Pathol 2011;6:65)

Treatment
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• Early surgery, but survival >5 years is rare

Clinical images
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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

Foot

Glistening erythematous ulcerated plaque

Nose

Gross description
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• Early: small, sharply demarcated, asymptomatic, multiple red nodules
• Late: fleshy, gray-white with hemorrhage, necrosis, deeply invasive

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

Ovary

Spleen

Thyroid

Micro description
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• 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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Breast

   

Lung: metastatic tumor

   

Lung: metastatic tumor

Nose

     

Ovary

 

Site unknown

 

Skin (image on right is epithelioid)

   

Spleen

       

Thigh mass, contributed by: Dr. Deepa Santhosh, Banaras Hindu University (India)

   

Thyroid gland

Spindle cell type, scalp

Spindle cell type, Fli-1 stain

 

Scalp

Granular cell type

 

Face

Epithelioid type, skin

 

Epithelioid type

 

Epithelioid type, face

Epithelioid, deep soft tissue

Cytology images
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Kidney

Positive stains
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• Factor 8 related protein, CD31, Ki-67, FLI-1 (nuclear stain, Am J Surg Pathol 2001;25:1061), thrombomodulin, CD34 (not specific), c-kit (50%)
• VEGFR-3 (variable, Mod Pathol 2000;13:180)
• keratin for epithelioid tumors

Molecular / cytogenetics description
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• Associated with high level amplifications of MYC, particularly in secondary tumors (Am J Pathol 2010;176:34, Genes Chromosomes Cancer 2011;50:25), but also in subset of primary tumors (Genes Chromosomes Cancer 2012;51:569)

Differential diagnosis
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• Atypical vascular lesions: circumscribed, no atypia, no mitotic figures
• Florid vascular proliferation of colon due to intussusception (Am J Surg Pathol 2001;25:1167)
• Hemangioma: usually < 2 cm, well circumscribed, contain fibrous septa and thick-walled vessels, not invasive
• MFH: intratumoral macrophages from non-vascular tumors may be CD31+, (Mod Pathol 2001;14:1114)

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