• 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

• 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%

• 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

• Unusual neoplasm
• <15 well documented cases of primary renal angiosarcoma

• Associated with arsenic, Thorotrast, PVC
• Latent period of years
• Also associated with radiation to other sites, introduction of foreign material

• 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)

• 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

• Rare neoplasm that often remains asymptomatic until massive intra-abdominal bleeding

• Small intestine
• Ovary

• 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)

• Early surgery, but survival >5 years is rare

Foot

69 year old man with raised lesion on nose

Nose

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

Ovary

Spleen

Thyroid

• 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

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

Kidney

• 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

• 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)

• 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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