Angiosarcomas are composed of a malignant population of endothelial-type cells forming complex anastomosing channels. The lumens are filled with red blood cells.

High-grade angiosarcomas can form solid sheets of epithelioid-appearing cells. Immunohistochemical studies may be necessary to distinguish recurrent carcinoma from radiation-induced angiosarcoma.



  • Angiosarcoma (AS)


  • Malignant neoplasm arising from endothelial cells or their precursors



  • Breast is most common site of origin of AS

    • However, AS are rare tumors compared to carcinomas (< 0.1% of all breast malignancies)


  • Women who have received radiation treatment for breast cancer have 9x increased risk of AS

    • However, absolute number of affected women is small: Approximately 3 in 1,000 women will develop AS after treatment at 15 years

    • Women who received chest wall radiation for Hodgkin disease are at increased risk for developing breast cancer, but AS has not been reported

      • Reason for this is unknown; probably related to age when radiated &&/or radiation doses

  • Incidence of tumors peaks from 5-10 years after treatment and is elevated out to 30 years


  • Women with markedly edematous arms after breast and axillary surgery for cancer are at increased risk for AS of skin (Stewart-Treves syndrome)

  • With more limited axillary surgery and better surgical techniques, this type of AS has become exceedingly uncommon



  • Age

    • Average age of women diagnosed with idiopathic AS: 40s

    • Average age of women diagnosed with radiation-related or edema-related AS: 60s

  • Gender

    • Almost all patients are female; male breast AS is very rare


  • Idiopathic AS generally arises deep in the breast

    • Large tumors may also involve the skin

  • Radiation-related AS arises in the skin

    • Tumors involve deeper breast tissue if large

    • Atypical vascular lesions (AVLs) also occur in the skin in this group of patients

    • Distinguishing AS from AVL may be difficult in some cases

  • Edema-related AS arises in the skin of edematous area

    • Arm is most common site


  • Patients with radiation-related or edema-related AS present with dark red or violaceous discoloration of skin

    • Palpable mass may be associated with skin changes

  • Patients with idiopathic angiosarcoma present with ill-defined palpable mass in the breast


  • Most effective treatment is surgical removal of tumor with wide margins

    • Most patients will require mastectomy to achieve this goal

  • Chemotherapy and radiation therapy are also used but have uncertain benefit


  • Many patients develop distant metastases

    • Most common sites are lung, liver, bone, and contralateral breast

    • Metastasis to regional lymph nodes is rare

  • Median survival: 3 years

    • Same for idiopathic and treatment-related AS

  • Larger tumors have poorer prognosis

    • Tumor grade has not been consistently linked to prognosis


Mammographic Findings

  • Tumors may be difficult to image as desmoplastic response commonly associated with carcinomas is not present

Ultrasonographic Findings

  • Tumors may appear lobulated, irregular, or ill defined

  • Echogenicity may be variable

    • May have hyperechoic areas if AS invades into adipose tissue



  • Tumors vary in size

    • Range: 1-20 cm

    • Average: 5 cm

    • Majority > 2 cm

      • Almost all vascular lesions of the breast > 2 cm in size will be malignant

  • Size can be difficult to determine grossly both visually and by palpation

    • Central portion of the tumor is usually hemorrhagic and firm to palpation

    • Infiltrative periphery of the tumor can blend into surrounding tissue and may not be visible

Jul 6, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiosarcoma

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