Teaching Case

A 65-year-old female patient consulted for right breast mass accompanied by cutaneous thickening which had developed insidiously in the last few months. She had a previous history of right breast cancer treated with lumpectomy and axillary dissection followed by radiotherapy and tamoxifen eleven years ago.

A 65-year-old female consulted for right breast mass accompanied by cutaneous thickening which had developed insidiously in the last few months.
She had a previous history of infiltrating ductal carcinoma (stage I, SBR grade I) of that same breast treated with lumpectomy and axillary dissection followed by radiotherapy and tamoxifen, eleven years previously.
A family history of breast and ovary cancer was reported in two of her sisters, suggesting an underlying genetic predisposition.
A mass was palpable in the retroareolar region of the right breast accompanied by reddish and purple papules in the internal inferior quadrant.
Mammography and ultrasound confirmed a 25 mm retroareolar mass of the right breast. The mass was heterogeneous, without evident internal vascularisation or posterior acoustic shadowing.
Magnetic resonance imaging (MRI) depicted a lobulated mass in the union of all quadrants disrupting and deforming the nipple–areolar complex, associated with cutaneous thickening (Fig. 1-4). The mass enhanced heterogeneously and cutaneous foci of early enhancement and rapid washout were observed (Fig. 2). Reduction of right breast volume due to breast conservative treatment was also noted (Fig. 4).
The hypodermal and dermal changes in the clinical setting of breast discoloration, cutaneous thickening and previous history of breast conservation therapy with radiotherapy made plausible the possibility of secondary angiosarcoma. Biopsy confirmed the diagnosis of high grade angiosarcoma (Fig. 5).
In the staging work-up, lung metastases were discovered (Fig. 6). The patient was treated with right mastectomy and doxorubicine.

Angiosarcoma is a rare and aggressive endothelial malignancy. The breast is one of angiosarcoma’s most common locations, accounting for 0,04% of breast’s malignancies and 8% of breast’s sarcomas.

Primary and secondary angiosarcoma are distinguished by the association of the latter with risk factors, namely chronic lymphedema and radiotherapy. Long-lasting lymphedema-associated limb and chest wall angiosarcoma was first described by Stewart and Treves, constituting a rare but typical syndrome observed after mastectomy and complete axillary lymph node dissection in radiation-naive patients. Thereafter, others causes of lymphedema-associated angiosarcoma have been described, including congenital, idiopathic, filariasis, and traumatic. In contradistinction to Stewart-Treves syndrome, postirradiation angiosarcoma is rare following mastectomy, generally occurring after breast-conserving therapy, with estimated prevalence of 0,9-1,59 cases/1000 and relative risk of 15,9.

Average latency period after radiation is 6 years (range 1-41 years). Therefore, secondary angiosarcoma tends to arise latter than primary, the mean age at diagnosis being 65 years. It usually affects the dermis and hypodermis within the radiation field but may also develop in breast parenchyma. Mean tumour size is 7,5 cm (range 0,4–20 cm). Multicentricity is seen in a third of patients.
Angiosarcoma may be asymptomatic or manifest by breast enlargement, a palpable mass (ranging from small cutaneous nodules to involvement of the entire breast), and/or skin changes which can be its only sign and comprise thickening, discoloration, macules, papules, or vesicles.

Mammograms are nonspecific, they may be normal (19-33% of cases) or portray a focal asymmetry as well as an ill-defined or circumscribed, single or multifocal masses. Calcifications are rare.

Ultrasound and MR imaging are capable of demonstrating mammographically occult angiosarcomas. Sonographic findings may vary and be nonspecific but a heterogeneous, mixed or predominantly hyperechogeneous, hypervascularized mass or architectural distortion should alert for the possibility of angiosarcoma. These features have been attributed to the vascular, infiltrating, fatty, and haemorrhagic nature characteristic of angiosarcoma.

MRI is useful in determining loco-regional extent, planning surgery, and detecting residual disease after incisional biopsy. Angiosarcoma often appears as lobulated, intensely and heterogeneously enhancing mass with rapid enhancement and washout kinetics, possibly with haemorrhage. In the right clinical setting, independent of the size, an enhancing skin focus in the postoperative and irradiated site should arise the diagnosis of cutaneous angiosarcoma.

Angiosarcoma’s prognosis depends on the histologic grade and extent, with a median survival of 14.5-37 months and a 5-year survival of 15%. With increasing use of breast-conserving therapy, reports of postirradiation angiosarcoma have increased. Nevertheless, it remains a rare entity that can easily be overlooked and have a late diagnosis due to the non rare lack of specificity of clinical and mammographic findings. The radiation induced skin changes are alternative or concurrent conditions, which can mask or mimic angiosarcoma, adding to its diagnostic complexity. Therefore, a high grade of suspicion is needed for early and appropriate diagnosis. The case presented demonstrates that in this high risk group, a mass with preferential dermal and hypodermic location associated to an enhancing skin focus should make us consider this rare but increasingly relevant malignancy.