Abstract

BackgroundPrimary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. Optimal management is mostly based on expert opinion. Our study aims to describe a single institution experience with breast angiosarcoma and evaluate other publications on this topic to further clarify prognostic outcomes and treatment modalities in this disease.MethodsTwenty two cases of breast angiosarcoma from Roswell Park Comprehensive Cancer Center were retrospectively analyzed. Additionally, a systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS).Results9 PAS patients (41%) and 13 SAS patients (59%) were retrospectively analyzed. No significant differences were noted in tumor characteristics and survival outcomes between PAS and SAS. Treatment modality had no significant effects on survival outcomes although adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. 380 PAS and 595 SAS patients were included in the outcome meta-analysis. Survival outcomes were significantly worse with high grade tumors and tumor size of > 5 cm. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes.ConclusionTumor size and grade seem to be reliable predictors of survival in both PAS and SAS. Mastectomy does not seem to be adding any additional benefit to BCS. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors.

Highlights

  • Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature

  • 22 patients were identified with breast angiosarcoma, 9 (41%) patients were diagnosed with primary angiosarcoma (PAS), and 13 (59%) patients with secondary breast angiosarcoma (SAS)

  • Breast angiosarcoma is a rare aggressive tumor characterized by high grade and high rate of local recurrence

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Summary

Introduction

Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. A systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS). Treatment modality had no significant effects on survival outcomes adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors. Breast angiosarcoma can be divided into two categories: primary (de novo) and secondary (therapy-related). Breast primary angiosarcoma (PAS) develops de novo with no prior breast radiation.

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