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WikiDoc Resources for Angiosarcoma


Most recent articles on Angiosarcoma

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US National Guidelines Clearinghouse on Angiosarcoma

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Risk calculators and risk factors for Angiosarcoma

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Symptoms of Angiosarcoma

Causes & Risk Factors for Angiosarcoma

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Treatment of Angiosarcoma

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List of terms related to Angiosarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Mohsen Basiri M.D.
Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma


Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls. The peak age of incidence appears to be the 7th decade, and men are affected more commonly than women. Angiosarcoma was first described by Dr. Juan Rosai, in 1976. The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular endothelial cells. Angiosarcoma may arise in any part of the body, but is more common in soft tissues than in bones. Common angiosarcoma locations include the head and neck area, kidney, liver, lung, and and the most common site of radiation-induced angiosarcoma development is the breast. The PTPRB/PLCG1 genes are associated with the development of angiosarcoma; mutation of these genes result in aberrant angiogenesis. The imaging modality of choice for diagnosing angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast. For other types angiosarcoma, the imaging modality of choice is MRI. On CT scan, findings suggestive of angiosarcoma may include vascular invasion, nodular enhancement (common), and a hypoattenuating mass. The mainstay adjuvant therapy for angiosarcoma is a doxorubicin-based regimen. The response rate for chemotherapy in patients with angiosarcoma is poor.

Historical Perspective

  • Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.[1]


  • Angiosarcoma may be classified according to the clinical heterogeneity into two main groups, and every group can be subdivided into subtypes according to the anatomical location and etiology:[2][3][4]
Primary Secondary
Cutaneous Post Radiation Angiosarcoma
Breast Lymphedema-associated Angiosarcoma
Soft tissue and Bone Angiosarcoma due to exposure to mutatgens
Visceral -


Image courtesy of Yale Rosen, contributed by Wikimedia commons


Differentiating Angiosarcoma from Other Diseases

Epidemiology and Demographics

  • The incidence of angiosarcomas can be calculated approximately 1.2 per 1,000.000 person.[5][18]
  • Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.The peak age of incidence appears is the 7th decade,[19]
  • Males are more commonly affected with angiosarcoma than females.[19]
  • The male to female ratio is 2:1.[19]
  • There is no racial predilection for angiosarcoma. however, African-Americans in the U.S are rarely affected.[20]

Risk Factors

Common risk factors in the development of angiosarcoma include chronic lymphedema, chronic exposure to polyvinyl chloride (PVC), radiation exposure, and exposure to Thorotrast.[5]

Natural History, Complications and Prognosis


Diagnostic Study of Choice


Stage Grade Site Metastasis
Ia Low grade (G1) Intracompartmental (T1) No metastasis (M0)
Ib Low grade (G1) Extracompartmental (T2) No metastasis (M0)
IIa High grade (G2) Intracompartmental (T1) No metastasis (M0)
IIb High grade (G2) Extracompartmental (T2) No metastasis (M0)
IIIa Low or High grade (G1-G2) Intracompartmental (T1) Metastasis (M1)
IIIb Low or High grade (G1-G2) Extracompartmental (T2) Metastasis (M1)

History and Symptoms

  • Angiosarcomas occur at different anatomic sites and grow insidiously, then they can present with various misleading symptoms.[24]
  • The most common clinical manifestation is a gradually enlarging, painless mass.[21]
  • Some patients complain of pain or symptoms due to compression of adjacent neurovascular structures that causes pain or edema in an extremity.
  • Secondary angiosarcomas include radiation-Induced and lymphedema-associated angiosarcoma have a distinct feature, presenting as single or several ecchymotic maculopapular cutaneous lesions in the radiation field or in areas exposed to chronic lymphedema.[25]

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with angiosarcoma.


Echocardiography or Ultrasound


Imaging Findings

  • The imaging modality of choice for angiosarcoma will depend on the location.
  • For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast.[19]
  • For other types angiosarcoma, the imaging modality of choice is MRI.

CT Scan

On CT, findings of angiosarcoma may include vascular invasion, nodular enhancement (common), and multicentric lesions.[19]


Other Imaging Findings

  • There are no other imaging findings associated with angiosarcoma.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with angiosarcoma.


Medical Therapy


  • The mainstay of treatment for angiosarcoma is complete surgical resection with wide margins for local and locoregional disease in combination with preoperative or postoperative radiotherapy.[34][35]
  • Surgical resection in combination with radiation therapy is the treatment of choice for small angiosarcomas.[5]
  • Complete surgical resection with wide margins is preferred for local and locoregional angiosarcoma.[5]
  • Owing to the tendency for local infiltration, surgical resection should be associated with preoperative or postoperative radiotherapy.[36]
  • Surgery is not recommended on patients with large sized angiosarcomas.
  • It usually occurs after a median of six months locally or distantly and the three-year disease-free and overall survival rates both are low.[37][38]

Primary Prevention

Secondary Prevention


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