Primary Angiosarcoma of the Breast: A Single-Center Retrospective Study in Korea

Abstract

Due to the rarity of primary angiosarcoma of the breast, optimal management is based on expert opinion. The aim of this study was to review all primary angiosarcomas of the breast obtained from a single center in terms of clinicopathologic characteristics, treatment, and survival outcomes. From 1997 to 2020, 15 patients with primary angiosarcoma of the breast underwent either mastectomy or wide excision. We analyzed the clinicopathologic data to assess disease-free survival and overall survival. Fifteen women with primary angiosarcoma of the breast were identified. The mean age at diagnosis was 33 years (range: 14-63 years). The overall mean tumor size was 7.7 cm (range 3.5-20 cm). Upon histological grading, there were three cases of low grade, five intermediate grade, six high grade, and one unidentified grade. The five-year disease-free survival rate was 24.4%, and the five-year survival rate was 37.2%. The survival rate of the low-grade patient group was statistically higher than that of the intermediate- or high-grade patient groups (p = 0.024). Primary angiosarcoma of the breast is a rare aggressive tumor characterized by high grade and poor outcome. Histologic grade appears to be a reliable predictor of survival. There are no standard treatment guidelines; thus, optimal R0 surgical resection remains the best approach. The roles of neoadjuvant, adjuvant chemotherapy, and radiotherapy remain unclear.

Keywords: angiosarcoma; breast angiosarcoma; primary angiosarcoma of the breast; primary sarcoma.

Figure 1

Disease-free survival and overall survival of primary angiosarcoma of the breast. The 5-year disease-free survival rate was 24.4% and the 5-year survival rate was 37.2%.

Figure 2

Overall survival according to tumor size. The 5-year survival rate was 28.3% in the group with tumor size ≥5 cm and 66.7% in the group with tumor size <5 cm. There was no significant difference between groups (p = 0.096).

Figure 3

Overall survival by tumor grade. The 5-year survival rate was 100% in the low-grade group, 30% in the intermediate-grade group, and 0% in the high-grade group. The survival rate of the low-grade group was significantly higher than that of the intermediate- and high-grade groups (p = 0.024).

Figure 4

Overall survival (A) and disease-free survival (B) according to adjuvant treatment. There were no significant differences in 5-year overall survival (p > 0.05, Figure 4A) and 5-year disease-free survival (p > 0.05, Figure 4B) between groups according to adjuvant treatment. Abbreviations: adjuvant Tx, adjuvant chemotherapy; RT, radiotherapy; chemo, chemotherapy; neoadjuvant chemo, neoadjuvant chemotherapy.