angiosarcoma of breast

Two cases of
radiation-associated angiosarcoma of the breast. Imaging findings (case 1). a Mammography. b Breast ultrasound. c Computed tomography. d Magnetic resonance imaging. Arrow: tumor
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A case of
primary breast angiosarcoma with multiple discontinuous small lesions. Doppler sonography showed hypervascularization of the tumor
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A case of
primary breast angiosarcoma with multiple discontinuous small lesions. MRI showed a gradually enhanced, well-circumscribed tumor
surgicalcasereports.springeropen.com • CC-by-4.0
Recurring
radiation-induced angiosarcoma of the breast that was treated with paclitaxel chemotherapy: a case report. a PET/CT before the surgery showed no metastatic disease. b PET/CT showed accumulation in the tumor
surgicalcasereports.springeropen.com • CC-by-4.0
Recurring
radiation-induced angiosarcoma of the breast that was treated with paclitaxel chemotherapy: a case report. a Ultrasonographic images of the patient with the tumor. The inside of the tumor had a low echoic area, and the tumor borders were unclear. b The tumor grew larger 2 months later
surgicalcasereports.springeropen.com • CC-by-4.0
Primary
breast angiosarcoma: a case report. a–d Ultrasound showed a large irregular mixed echogenic mass with solid and cystic components within seen occupying almost the entire breast. Multiple large vessels with turbulent flow and pulsations were seen within this mass (arrows)
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Primary
breast angiosarcoma: a case report. MRI of the breast revealed a large low T1 (a), high T2 (b) signal intensity mass (thin arrow) occupying the entire left breast with ‘blood lake’ (thick arrow) within. It demonstrated restricted diffusion on DWI and ADC (c, d), rapid enhancement post-contrast administration with progressive and prolonged enhancement in the delayed phase (e, f)
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Multimodality
approach to the nipple-areolar complex: a pictorial review and diagnostic algorithm. Radiation-associated angiosarcoma. A 76-year-old woman with a history of breast cancer treated with conservative surgery and whole-breast radiation therapy 6 years prior. a Photograph shows an ill-defined erythematous-violaceous infiltrated plaque with an eroded area occupying part of the areola. b Synthesized 2D mammogram shows skin thickening and interstitial edema in the retroareolar region of the left breast. c Ultrasound shows an ill-defined hypoechoic skin lesion with an internal Doppler signal. Punch biopsy diagnosed radiation-associated angiosarcoma, and the patient underwent mastectomy
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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.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Angiosarkom der Mamma:
Mammakarzinom
 
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Angiomatose
der Mamma
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