Primary Angiosarcoma Of the Breast: A Case Report

Abstract

Primary angiosarcoma of the breast is a rare (0.04% of all malignant breast tumors) and potentially life-threatening disease. Given its variable and non-specific clinical, radiological and pathological presentation, accurate diagnosis is a challenge. Primary angiosarcoma of the breast predominantly occurs in younger patients and it is often overlooked and misdiagnosed at radiology and pathology. To ensure that this aggressive malignancy is not overlooked, radiologists need to be aware of the fact that such tumors may present with non-specific imaging features. We report a case of a 32-year-old female with primary angiosarcoma of the breast presenting with non-specific imaging features. It was initially interpreted as a capillary cavernous hemangioma at histopathology following an ultrasound-guided biopsy. This eventually turned out to be angiosarcoma after a second histopathology opinion was sought in light of the radiology-pathology discordance.

Keywords: Angiosarcoma; Breast; Magnetic Resonance Imaging; Mammography; Primary; Ultrasonography.

Figure 1

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: A diffuse area of altered echotexture containing multiple hypoechoic tubular lesions (arrow in Figure 1a). No internal vascularity on color doppler image (arrow in Figure 1b). TECHNIQUE: (1a) Grey-scale ultrasound examination of the right breast performed with 9 MHz linear transducer. (1b) Color doppler performed with 9 MHz linear transducer.

Figure 2

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: Subcutaneous and parenchymal edema in the right breast (white dashed line) compared with normal subcutaneous fat in the left breast (yellow dashed line). TECHNIQUE: Grey-scale ultrasound examination performed with 9 MHz linear transducer.

Figure 3

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: Global asymmetry in the right breast (arrow in Figures 3a and 3b). Prominent vessels adjacent to pectoralis muscle (dashed arrows in Figure 3b). TECHNIQUE: 2D full field digital mammography performed with KVP 30 and exposure 160 mAs. Anode-tungsten, filter-rhodium. Bilateral cranio-caudal (a) and medio-lateral oblique (b) views.

Figure 4

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: No discrete mass on 3D digital breast tomosynthesis. Mild skin thickening of the right breast (arrow). TECHNIQUE: 3D full-field digital breast tomosynthesis performed with KVP 30 and exposure 420–480 mAs in cranio-caudal and medio-lateral oblique views. Anode-tungsten, filter-rhodium. Slice thickness 1 mm.

Figure 5

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: An irregular mass showing isointense signal on T1W images (white arrow in Figure 5a) and hyperintense signal on T2W images (white dashed arrow in Figure 5b) involving most of the upper quadrant of the right breast. Mild cutaneous thickening of the right breast (yellow arrow in Figure 5a). TECHNIQUE: 1.5 Tesla magnet (a) Axial MRI T1W sequence. Slice thickness 1.4 mm, TR 14, TE 4.7. (b) Axial MRI T2W sequence. Slice thickness 4 mm, TR 3600, TE 66.

Figure 6

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: Focal area of restricted diffusion in superomedial part of the mass. High signal intensity on diffusion weighted images (DWI) (white arrow in Figure 6a) and low signal intensity on ADC (Apparent Diffusion Coefficient) parametric map (yellow arrow in Figure 6b). TECHNIQUE: 1.5 Tesla magnet (a) DWI with b values 0 and 800 sec/mm2. Slice thickness 5 mm, TR 5200, TE 82 (b) ADC map. Slice thickness 5 mm, TR 5200, TE 82.

Figure 7

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: An irregular heterogeneously enhancing mass in upper quadrant of the right breast during early phase (90 seconds) post-contrast fat-suppressed subtracted axial T1W image (white arrow in Figure 7a) and during late phase post-contrast fat-suppressed subtracted T1W image (white dashed arrow in Figure 7b). Same mass seen in MIP (Maximum Intensity Projection) images at the level of nipple during early phase post-contrast fat-suppressed subtracted T1W image (white block arrow in Figure 7c) and during late phase post-contrast fat-suppressed subtracted T1W image (white curved arrow in Figure 7d). Prominent internal mammary vessels observed along the chest wall extending towards inner quadrant of the right breast (yellow arrow in Figure 7b). TECHNIQUE: 1.5 Tesla magnet. Dynamic post-contrast fat-suppressed subtracted axial T1W images. 0.1 mmol/kg intravenous contrast Gadopentetate dimeglumine injected at the rate of 2ml/second followed by a 20 ml normal saline flush (a) during early phase (90 seconds) in upper quadrant of the right breast, slice thickness 1.1 mm, TR 4.2, TE 1.5 (b) during late phase in upper quadrant of the right breast, slice thickness 1.1 mm, TR 4.2, TE 1.5 (c) MIP image at the level of nipple during early phase (d) MIP image at the level of nipple during late phase.

Figure 8

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: A large irregular enhancing mass involving most of the upper quadrant of the right breast (arrow in Figure 8a). The normal left breast for comparison (Figure 8b). TECHNIQUE: 1.5 Tesla magnet. Sagittal MRI T1W fat-suppressed post-contrast sequence of the right breast (Figure 8a) and the left breast (Figure 8b). 0.1 mmol/kg intravenous contrast Gadopentetate dimeglumine injected at the rate of 2ml/second followed by a 20 ml normal saline flush. Slice thickness 1.2 mm, TR 4.2, TE 1.5.

Figure 9

32-year-old Female Patient with Primary Angiosarcoma of the Right Breast. FINDINGS: (a) An irregular mass composed of complex anastomosing vascular channels (black block arrow in figure 9a). (b) Infiltration of the tumor into and surrounding the adipose tissue (Red arrow in Figure 9b) while a benign duct also seen (yellow arrow in Figure 9b). (c)Vascular channels lined by atypical and proliferated endothelial cells with minimal cellular and nuclear pleomorphism (black dashed arrow in Figure 9c). TECHNIQUE: Hematoxylin and eosin stain (a) Low power view (b) Intermediate power view (c) High power view.