Angiosarcoma of the Breast: Overview of Current Data and Multimodal Imaging Findings

Abstract

Angiosarcoma of the breast is a rare breast cancer, which can arise de novo (primary breast angiosarcoma, PBA) or as a secondary malignancy (secondary breast angiosarcoma, SBA) as a result of a biological insult. In the latter case, it is usually diagnosed in patients with a previous history of radiation therapy following a conserving treatment for breast cancer. Over the years, the advances in early diagnosis and treatment of breast cancer, with increasing use of breast-conserving surgery and radiation therapy (instead of radical mastectomy), brought about an increased incidence of the secondary type. PBA and SBA have different clinical presentations and often represent a diagnostic challenge due to the nonspecific imaging findings. The purpose of this paper is to review and describe the radiological features of breast angiosarcoma, both in conventional and advanced imaging to guide radiologists in the diagnosis and management of this rare tumor.

Keywords: angiosarcoma; breast imaging; computed tomography; magnetic resonance imaging; mammography; primary breast angiosarcoma; prognosis; secondary breast angiosarcoma; treatment; ultrasound.

Figure 1

An 85-year-old patient with a history of carcinoma of the right breast treated with quadrantectomy and radiotherapy. (a–c) Clinical examination revealed papillary erythematous lesion, skin thickening, and multiple reddish nodules.

Figure 2

A 60-year-old patient with a history of gastric lymphoma treated 30 years previously. Clinical examination revealed an erythematous area on the right breast. (a) Craniocaudal and (b) mediolateral oblique mammograms of the right breast show an irregular mass. A core needle biopsy was performed with the simultaneous placement of a clip marker (white dot) and the presence of a primary BA was histologically proven.

Figure 3

A 70-year-old patient with a history of carcinoma of the left breast treated with quadrantectomy and radiotherapy. Three years after completion of radiotherapy, clinical examination revealed a reddish cutaneous area and a palpable mass in the surgical scar location. (a) Craniocaudal and (b) mediolateral oblique mammograms of the left breast show a high density, irregular mass below the scar associated to skin thickening. The white lines represent the surgical clips released at the time of the quadrantectomy.

Figure 4

A 58-year-old patient with a history of carcinoma of the left breast treated with quadrantectomy and radiotherapy. Seven years after completion of radiotherapy, clinical examination revealed an erythematous lesion in the scar location. (a) Craniocaudal and (b) mediolateral oblique mammograms of the left breast show only skin thickening without mass or calcifications association. Due to the persistence of the cutaneous lesion, a punch biopsy was performed revealing an SBA.

Figure 5

A 75-year-old patient with a history of lobular invasive carcinoma of the right breast treated with quadrantectomy and radiotherapy. Clinical examination revealed skin discolouring and teleangectasis five years after completion of radiotherapy. (a) Right craniocaudal (RCC) and (b) mediolateral oblique (MLO) mammograms show scar remodeling and skin thickening. No mass or suspicious calcifications were detected. A histological diagnosis of SBA was made with a punch biopsy.

Figure 6

A 60-year-old patient with a palpable mass. US image of a PBA characterized by a voluminous heterogeneous hyperechoic mass, with non-circumscribed margins, with associated skin thickening.

Figure 7

A 58-year-old patient with a history of carcinoma of the left breast treated with quadrantectomy and radiotherapy. US image of an SBA characterized by a diffuse skin thickening and an oval-shaped hypoechoic lesion with circumscribed margins.

Figure 8

A 75-year-old patient with a history of lobular invasive carcinoma of the right breast treated with quadrantectomy and radiotherapy. US image of an SBA characterized by skin thickening and an irregular mass with indistinct margins in the retroareolar area. A US-guided core needle biopsy was performed, revealing a single focus of atypical cellular proliferation. Then, a punch biopsy was performed and an SBA was histologically proven.

Figure 9

A 60-year-old patient with PBA. (a) T2-weighted, (b) axial 3D gradient echo T1-weighted post-contrast and (c) sagittal 3D gradient echo T1-weighted post-contrast images show an irregular mass, with non-circumscribed margins, characterized by heterogeneous enhancement, with skin thickening and invasion.

Figure 10

A 58-year-old patient with a history of carcinoma of the left breast treated with quadrantectomy and radiotherapy. (a) T2-weighted and (b) 3D gradient echo T1-weighted post-contrast images show skin thickening near the scar and late contrast enhancement. Punch biopsy revealed SBA.

Figure 11

A 48-year-old patient with a history of left breast cancer treated with quadrantectomy and radiotherapy. (a) T2-weighted, (b) axial 3D gradient echo T1-weighted post-contrast and (c) sagittal 3D gradient echo T1-weighted post-contrast images show skin thickening and an oval-shaped mass, with circumscribed margins, characterized by homogenous enhancement. US-guided core needle biopsy reveals SBA.

Figure 12

A 65-year-old patient with a history of PBA treated with right mastectomy, right axillary lymph node dissection and radiotherapy of the right axilla. A 2-year follow-up with CT scan revealed metastatic disease: (a) Mediastinal window showing a voluminous mass in the left breast, an enlarged lymph node in the right axilla, and a nodular thickening in the subcutaneous soft tissues of the right arm and of the left lateral chest wall. (b) Parenchymal window showing numerous bilateral pulmonary nodules.

Figure 13

A 75-year-old patient with a history of lobular invasive carcinoma of the right breast treated with quadrantectomy and radiotherapy. Mediastinal window of a follow-up CT scan shows skin thickening of the right breast.

Figure 14

A 65-year-old patient with a history of PBA treated with right mastectomy, right axillary lymph node dissection, and radiotherapy of the right axilla. A 2-year follow-up with ¹⁸F FDG PET-CT scan revealed metastatic disease: (a,b) hypermetabolic lesions are observed in breasts, lungs, and bones.