Angiosarcoma (AS) of the Breast

• Angiosarcoma (AS) of the breast is rare:
  • Accounting for far less than 1% of all soft tissue breast tumors.
• It presents as a primary tumor of the breast or as a secondary lesion most commonly associated with previous radiotherapy:
  • Primary AS has been observed in women ages 30 to 50 years presenting with poorly defined masses.
  • It accounts for less than 0.04% of malignant neoplasms.
  • Typically arises in the parenchyma of the breast, and has occasional skin involvement.
  • Women with primary AS usually present with a palpable mass, fullness or swelling in the breast:
    • Which at times can be rapidly growing.
• In contrast, secondary AS presents in older women (median age 67 to 71 years) following a median of 10.5 years after radiotherapy for breast cancer:
  • The median latency to presentation after radiotherapy in 7 series ranges from 5 to 10 years.
  • Although a causal relationship between radiation exposure and AS has not been established, multiple case reports support the increased risk for AS following adjuvant radiotherapy:
    • It has been proposed that at radiation doses greater than 50 Gy, apoptosis occurs while at less than 50 Gy DNA damage and instability result.
  • Sarcomas frequently occur at the edge of radiation fields where doses and tumor necrosis may be heterogeneous.
  • When associated with chronic lymphedema and located outside a radiated field:
    • AS in an edematous limb after mastectomy and radiotherapy is referred to as Stewart–Treves syndrome.
  • Secondary AS presents as painless bruising that is frequently multifocal but can present with a mass:
    • It is often neglected because of its seemingly innocent appearance.
    • There are other varied descriptions of the presenting signs including :
      • Purplish discoloration, eczematous rash, hematoma-like swelling, and diffuse breast swelling.

• Kasabach–Merritt syndrome, also known as hemangioma with thrombocytopenia, is a rare disease in which vascular tumors lead to platelet sequestration and hemorrhage:
  • Although it occurs primarily in infants with hemangioma, rarely has it been reported in angiosarcomas.
• On histopathological analysis the lesions are notable for irregular vascular formations with hyperchromatic and irregular nuclei (Figure ):

• The diagnosis can be clarified by immunohistologic staining for the endothelial marker CD31 as in this case which determines the tumor is of endothelial origin:
  • CD31 is the most sensitive and specific indicator of angiogenic proliferation; however, the lesions will also stain positive for the vascular markers:
    • Factor VIII, and Fli1, and will usually at least be weakly positive for CD34.

 

Rodrigo Arrangoiz MS, MD, FACS a surgical oncologist and is a member of Sociedad Quirúrgica S.C at the America British Cowdray Medical Center in Mexico City:

• He is an expert in the management of breast cancer.

  • If you have any questions about angiosarcoma of the breast cancer  please fill free to contact Dr. Arrangoiz.

Training:

• General surgery:

• Michigan State University:

• 2004 al 2010

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• Fox Chase Cancer Center (Filadelfia):

• 2010 al 2012

• Masters in Science (Clinical research for health professionals):

• Drexel University (Filadelfia):

• 2010 al 2012

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• IFHNOS / Memorial Sloan Kettering Cancer Center:

• 2014 al 2016

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