Abstract
BackgroundBreast angiosarcoma is a rare malignancy with limited publications confined to small retrospective case reviews and case reports. Knowledge of this disease is limited because information from previous studies is insufficient and inconsistent.MethodsWe obtained data from the Surveillance, Epidemiology, and End Results Program for non-metastatic primary and secondary breast angiosarcoma, and performed analysis to determine clinicopathological characteristics and estimate their associations with overall survival (OS).ResultsMedian age was 50–54 years in primary breast angiosarcoma and 70–74 years in secondary breast angiosarcoma, while median OS was 93 and 32 months, respectively. Age, tumor grade and tumor spread were associated with poor survival outcomes. Compared with primary breast angiosarcoma, patients with secondary breast angiosarcoma had a “nominal” increased death risk (HR = 1.89, 95% CI, 1.43–2.50, p < 0.001), which was driven by older age and more aggressive tumor phenotype at presentation. Mastectomy was associated with worse OS compared with breast conservative surgery (BCS) (adjHR = 2.47, 95% CI, 1.29–4.74) in primary angiosarcoma patients. Adjuvant radiation was associated with worse OS in secondary angiosarcoma patients (adjHR =1.77, 95% CI, 1.01–3.12).ConclusionsThere is a “nominal” increased death risk in secondary breast angiosarcoma due to advanced clinicopathological features. Both BCS and mastectomy are feasible in primary and secondary angiosarcoma if R0 can be achieved. Routine radiation in unselected breast angiosarcoma should be cautious because there is no survival benefit in primary AS and appeared to be associated with a worse OS in secondary AS.
Highlights
Breast angiosarcoma is a rare malignancy with limited publications confined to small retrospective case reviews and case reports
Angiosarcoma constitutes about 31.7% of all primary breast sarcoma and about 70.9% of secondary breast sarcoma without metastasis. Those numbers were extrapolated by the percentage of angiosarcoma in breast sarcoma without or with prior cancer history, because it is difficult to determine the exact number of therapy-related breast sarcoma if patients ever received surgery or radiation
After applying the strict inclusion criteria as described in Methods, we identified a total of 218 non-metastatic primary breast AS patients and a total of 173 non-metastatic secondary breast AS patients for further analyses
Summary
Breast angiosarcoma is a rare malignancy with limited publications confined to small retrospective case reviews and case reports. Knowledge of this disease is limited because information from previous studies is insufficient and inconsistent. Of all the breast sarcomas, angiosarcoma (AS) is the main histology type and carries a poor prognosis. It is a very aggressive malignant tumor of the vascular endothelium, characterized by rapidly proliferating and extensively infiltrating growth. A literature search of Pubmed database yielded 79 primary breast AS and 26 secondary breast AS articles, most of which were small retrospective case reviews and case reports. Both radiotherapy and chemotherapy have been used, but treatment indications have
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