A case of primary breast angiosarcoma with multiple discontinuous small lesions

Abstract

BackgroundAngiosarcoma of the breast is rare. It carries a poor prognosis because of its high risk of local recurrence and distant metastases. Presently, there are still no established systemic therapies. Thus, the main treatment strategy for breast angiosarcoma is complete resection. This underscores the importance of closely monitoring the spread of the tumor lesion, particularly for multifocal angiosarcoma, and to plan an optimal operative procedure. We herein present the successful surgical treatment of a rare case of multifocal primary breast angiosarcoma.Case presentationA 43-year-old woman visited our hospital with a growing lump on her right breast accompanied by pain. Clinical and radiological examinations revealed a well-circumscribed 40-mm-diameter tumor at the inner lower quadrant of her right breast. Histological examination of a needle biopsy specimen revealed angiosarcoma. Based on a precise evaluation of the tumor by contrast-enhanced MRI and contrast-enhanced CT scan, a wide local excision with sufficient margins was performed. In the resected specimen, three discontinuous small lesions of angiosarcoma were observed around the main tumor. Therefore, total mastectomy was additionally performed. Pathological examination revealed two other small nodules of angiosarcoma in the remnant right breast, which appeared to be close but not continuous to the defective part of the initial resection. Postoperative follow-up at 1 year showed no signs of recurrence or distant metastasis. Multifocal primary breast angiosarcoma is extremely rare with only two previous reports describing its multifocality.ConclusionsOwing to its rarity, a standardized surgical treatment for breast angiosarcoma remains controversial. Our case suggests that primary breast angiosarcoma may occasionally present with multifocal tumor. Thus, it is important to keep in mind the multifocality of breast angiosarcoma when assessing its spread by diagnostic imaging and when planning the surgical strategy.

A rare presentation of pregnancy associated primary angiosarcoma of the breasts

“…It is an infiltrating overgrowth of vascular endothelial cells and is associated with hemorrhage and necrosis [12] , [13] , [14] . AS represents less than 1% of all primary breast cancers [1] , [2] , [3] and has an aggressive clinic course with an overall poor prognosis [10] . It can present as a primary cancer or a secondary form that is associated with a history of radiotherapy 10-year prior in older women (65-75 years) [9] .…”

“…Angiosarcoma (AS) of the breast is a rare mesenchymal tumor that accounts for less than 1% of all primary breast cancers [1] , [2] , [3] and 8% of all sarcomas. It has an annual incidence of 4.6-17 per 10 million women [4] .…”

Primary breast angiosarcoma resembling a common benign tumor: A case report

“…Total mastectomy with or without axillary node dissection is the preferred surgical treatment [ 13 ]. Metastases are usually hematogenous to the lungs, liver, bones, skin and the contralateral breast [ 14 ], and because of the rarity of breast angiosarcoma, randomized trials of adjuvant radiotherapy or chemotherapy are not yet available and the efficacy of this therapy still lacks evidence [ 15 ].…”

Use of extended field-of-view ultrasound imaging in giant primary breast angiosarcoma: a case description

“…The general treatment of primary breast angiosarcoma is total mastectomy (5), and adjuvant therapy may improve patient survival (1). The tumor size, histologic characteristics, and surgical margin are associated with prognosis of patients with primary breast angiosarcoma (17).…”

“…Well-differentiated angiosarcoma that is less than 5 cm in size, with a lower grade and a negative margin, tends to have a better prognosis (17).…”