Abstract / Summary
Abstract Background Primary breast angiosarcoma (PBA) is rare, and limited data exist because studies often combine primary and secondary angiosarcoma patients. Methods For patients with PBA who received care at a comprehensive cancer center from 1991 to 2025 and underwent surgical resection, this retrospective study collected and analyzed clinicopathologic features, treatment patterns, and survival data. Results Among 176 patients, 138 met the inclusion criteria. The median age was 40 years (interquartile range [IQR], 32–49 years). Most of the tumors were high grade with a median size of 6.5 cm. Most of the patients underwent mastectomy (88%). Among the 50 patients (36%) who underwent axillary surgery, only 6 patients had nodal metastases. Multimodal treatment was performed for 121 patients (88%), including chemotherapy/surgery ( n = 46, 38%), radiation therapy (RT)/surgery ( = 15, 12%), and chemotherapy/RT/surgery ( n = 60, 50%). The median follow-up time was 3.7 years. The median overall survival (OS) was 6 years (IQR, 3.5–8.7 years), and the 5-year OS was 58%. Recurrence was experienced by 62% of the patients, with a median recurrence-free survival (RFS) of 3 years (IQR, 1.4–7.7 years). Most of the recurrences were distant (79%), with 14% of the patients experiencing local recurrence only. Tumor size larger than 5 cm and high-grade histology were independently associated with lower OS. When stratified by treatment modality, there was no statistical difference in OS ( p = 0.475). Conclusions Primary breast angiosarcoma arises in women with a median age of 40 years, and large, high-grade tumors are associated with worse survival outcomes. Distant recurrence is common, whereas nodal metastases and local failure are not. Most of the patients in this study received multimodal therapy without a clear impact of modality on OS. However, further collaborative studies may be required to assess the impact of modality on disease outcomes.