Teaching Case

A 57-year-old woman diagnosed with invasive ductal carcinoma of the left breast in the past. She underwent quadrantectomy and lymph-node dissection followed by chemotherapy, radiotherapy and hormone therapy. She did well for 12 years. After this period stiffness of the left breast appeared with nipple retraction and flushing around the nipple.

Mammography (MG) and ultrasound (US) were immediately performed. 

MG revealed a dense condensation in the upper lateral quadrant of the left breast, thickened skin and nipple retraction (Fig. 1).

Breast US showed an irregular hypoechoic area in the central quadrant of the left breast with anechoic fluid components and increased vascularity (Fig. 2).

Breast MRI diagnosed a deformity of the left breast, thickened skin and nipple retraction. Centrally a large infiltrative lesion, hyperintense on T2-weighted images (Fig. 3a), hypointense on T1-weighted images (Fig. 3b), rapidly enhancing with 170% increase of SI (Fig. 3c) was present. The lesion showed kinetic curve Type-2 (Fig. 3e). The wash-in map pointed to high vascularity (Fig. 3f).

US-guided core-cut biopsy was performed and a diagnosis of high-grade secondary angiosarcoma of the breast was made.

Mammary angiosarcomas (AS) are exceedingly rare and highly aggressive breast tumours that occur in less than 0.05% [1]. AS appears de novo as a primary – in younger women without previous history of mammary carcinoma or any associated factor [4]; or as a secondary – most commonly associated with previous radiotherapy and occurs in older women (median age is 67-71 years) [2, 3]. The median onset latency after radiotherapy as treatment of primary breast cancer varies from 5-10 years [3]. Clinical presentation is different and may occur as a haematoma, reddish-purple, multifocal nodules, appearance of oedema,  swelling or eczematous rash [3].

MG, US, MRI, and ultrasound-guided core-cut biopsy are the key methods of correct and early diagnosis. However, the diagnosis of AS can be troublesome and delayed due to unclear and non-specific imaging findings especially on MG and US [4].  

The mammographic image is distorted by scarring and post-radiation changes of the breast as skin thickening, areolomammillar complex retraction, ill-defined lesions or condensations, and asymmetric gland distribution. Ultrasound shows only an irregular heterogeneous area usually with increased vascularity. MRI seems to be the best option for the determination of AS. It has a significant role in the assessment of the extent of the lesion. AS characteristically results in hyperintensity displayed on T2-images, hypointensity on T1 weighted images and a rapid post-contrast enhancement followed by wash-out. When an increase of signal intensity occurs in native T1-weighted images it is a manifestation of dilated vascular lacunes and bleeding zones in the tumour, typical for high-grade tumours.

The incidence of radiation-induced breast AS is increasing because breast-conserving treatment with radiotherapy has replaced mastectomy. 

Treatment is surgical, usually a radical mastectomy is performed followed by adjuvant chemotherapy.

Prognosis of breast AS is poor, even though the association of the radiotherapy and secondary AS is clear. Patients often present late and the disease has a rapid progression [4]. Diagnosis of AS can be sometimes missed, especially if only a small biopsy (FNAC) is taken, the histological diagnosis can be incorrect. Therefore a core-cut biopsy needs to be performed.

There is a necessity of follow-up after a multimodal treatment of breast cancer for early detection of the disease.