Table of Contents
Definition / general | Sites | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Positive stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Shankar, V. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueangiosarcoma.html. Accessed April 25th, 2018.
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
- 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
- Early surgery, but survival >5 years is rare
Clinical images
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Gross description
- Early: small, sharply demarcated, asymptomatic, multiple red nodules
- Late: fleshy, grayish white with hemorrhage, necrosis, deeply invasive
Gross images
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
- Post radiation lesions usually high grade
Microscopic (histologic) images
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Images hosted on other servers:
Images courtesy of Dr. Mark R. Wick:
Images hosted on other servers:
Contributed by Dr. Deepa Santhosh, Banaras Hindu University (India):
Images courtesy of Dr. Mark R. Wick:
Images hosted on other servers:
Contributed by Dr. Deepa Santhosh, Banaras Hindu University (India):
Positive stains
- Factor VIII related protein, CD31, Ki67, FLI1 (nuclear stain, Am J Surg Pathol 2001;25:1061), thrombomodulin, CD34 (not specific), c-kit (50%)
- VEGFR3 (variable, Mod Pathol 2000;13:180)
- Keratin for epithelioid tumors
Molecular / cytogenetics description
- 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
- 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)