Angiosarcoma of breast
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Breast angiosarcomas are a rare vascular breast malignancy.
As primary tumors of the breast, they account for ~0.04% 2 of all breast cancers and tend to occur in younger women, in their 3rd to 4th 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 6th decade 2).
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 9,10.
Patients may also present with swelling, a sensation of fullness, or rapid breast growth 10.
The tumors are of endovascular origin. There are two main types:
- primary angiosarcoma of the breast
- secondary angiosarcoma of the breast
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 11
- secondary breast angiosarcoma mainly involve the skin, with or without involvement of underlying breast parenchyma 11
- prior breast irradiation: radiation-induced breast angiosarcomas tend to occur after a significant latent period post-radiation (~5 years)
- Stewart-Treves syndrome
Lesions can sometimes be occult on mammography 7.
A heterogeneous mass is usually seen on MRI. Reported signal characteristics include 7:
- 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 12.
Surgical excision with wide margin is the standard of care, typically a mastectomy. Axillary nodal dissection is usually not performed, as the malignancy features hematogenous spread.
Despite aggressive surgery, there is a high local and distant recurrence rate.
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