Angiosarcoma of the breast: A difficult surgical challenge

Abstract

Primary angiosarcoma occurs de novo, presenting as a breast mass. Secondary angiosarcoma presents predominantly as a skin lesion, in the setting post-operative radiotherapy for breast conserving therapy. Angiosarcoma remains a rare and difficult management problem with poor loco-regional and distal control. Secondary AS is an iatrogenic condition that warrants close follow-up and judicial use of radiotherapy in breast conserving therapy.

Primary and Secondary Angiosarcoma of the Breast

“…In contrast, secondary AS presents in older women (median age 67-71 years) following a median of 10.5 years after radiotherapy for breast cancer (1,(4)(5)(6). The median latency to presentation after radiotherapy in seven series ranges from 5 to 10 years (1). Although a causal relationship between radiation exposure and AS has not been established, multiple case reports support the increased risk for AS following adjuvant radiotherapy (7)(8)(9).…”

“…There are few investigations and case reports that have examined the use of (neo)adjuvant paclitaxel, docetaxel, adjuvant vascular endothelial growth factor inhibitors, and hyperfractionated radiotherapy. These therapies show promise but need further examination (1,(33)(34)(35)(36)(37). Imatinib, a tyrosine kinase inhibitor has also been suggested as a beneficial treatment in radiation-induced sarcoma (38).…”

“…It accounts for <0.04% of malignant neoplasms and typically arises in the parenchyma of the breast with occasional skin involvement (1)(2)(3). In contrast, secondary AS presents in older women (median age 67-71 years) following a median of 10.5 years after radiotherapy for breast cancer (1,(4)(5)(6). The median latency to presentation after radiotherapy in seven series ranges from 5 to 10 years (1).…”

“…Angiosarcoma (AS) of the breast is rare, accounting for 1% of all soft tissue breast tumors (1). It presents as primary tumors of the breast or as secondary lesions that are most commonly associated with previous radiotherapy.…”

“…Sarcomas frequently occur at the edge of radiation fields where doses and tumor necrosis may be heterogeneous (4,5). When associated with chronic lymphedema and outside a radiated field, AS in an edematous limb after mastectomy and radiotherapy is referred to as Stewart-Treves syndrome (1,3).…”