Angiosarkom der Mamma

Breast angiosarcomas are a rare vascular breast malignancy.

Epidemiology

As primary tumors of the breast, they account for ~0.04% of all breast cancers and tend to occur in younger women, in their 3 to 4 decades.

Secondary angiosarcoma, related to prior therapy of breast cancer, has an estimated incidence of ~0.09-0.16% and occurs in older women (peak age 6 decade ).

Clinical presentation

The classic presentation is a painless localized blue or purple color change of the breast skin, with singular or multifocal lesions which may resemble other benign vascular lesions such as angioma or telangiectasis .

Patients may also present with swelling, a sensation of fullness, or rapid breast growth .

Pathology

The tumors are of endovascular origin. There are two main types:

  • primary angiosarcoma of the breast
  • secondary angiosarcoma of the breast
    • radiation-induced angiosarcoma: can occur following breast irradiation
    • lymphedema-associated cutaneous angiosarcoma
Location

Specific location of the tumor varies according to whether it is a primary or secondary lesion:

  • primary breast angiosarcoma are thought to arise within the breast parenchyma, with secondary involvement of the skin
  • secondary breast angiosarcoma mainly involve the skin, with or without involvement of underlying breast parenchyma
Associations
  • prior breast irradiation: radiation-induced breast angiosarcomas tend to occur after a significant latent period post-radiation (~5 years)
  • Stewart-Treves syndrome

Radiographic features

Mammography

Lesions can sometimes be occult on mammography .

Breast MRI

A heterogeneous mass is usually seen on MRI. Reported signal characteristics include :

  • T1: usually low signal intensity
  • T2: usually high signal intensity
  • C+ (Gd): low-grade lesions show progressive enhancement whereas high-grade lesions show rapid enhancement and washout

Treatment and prognosis

Angiosarcoma of the breast is an extremely aggressive tumor and is associated with a poor prognosis.

The tumor may be mis-diagnosed on the basis of fine-needle biopsy; thus, core biopsy is mandatory for diagnosis .

Surgical excision with wide margin is the standard of care, typically a mastectomy.  Axillary nodal dissection is usually not performed, as the malignancy features haematogenous spread.

Despite aggressive surgery, there is a high local and distant recurrence rate.

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