A Systematic Review on Breast Angiosarcoma

Breast angiosarcoma is an uncommon and aggressive mesenchymal malignancy originating from theendothelial cells of blood or lymphatic vessels. It accounts for a very small percentage of all breast cancersand soft tissue sarcomas. This malignancy manifests in two principal forms: primary angiosarcoma, whicharises de novo in the breast parenchyma, typically in younger women; and secondary angiosarcoma, whichis more common and usually develops in older women as a consequence of prior radiation therapy for breastcancer (radiation-induced angiosarcoma or RIBAS) or chronic lymphedema. Both forms are characterizedby aggressive clinical behavior and a generally poor prognosis. Diagnosis is often challenging due to variedand sometimes subtle clinical presentations, non-specific imaging findings, and pathological subtleties thatcan mimic benign vascular lesions or other malignancies. Histopathological examination withimmunohistochemical markers, particularly CD31 and MYC (for secondary forms), is crucial forconfirmation. Treatment predominantly involves surgical excision with the goal of achieving wide negativemargins, often a mastectomy. The roles of adjuvant radiotherapy, especially in RIBAS, and systemictherapies (chemotherapy, targeted therapy, immunotherapy) are still evolving and lack definitive consensusdue to the disease's rarity and paucity of large-scale prospective trials. Prognostic factors include tumorgrade, size, margin status, patient age, and tumor type (primary vs. secondary). High rates of local and distantrecurrence contribute to unfavorable survival outcomes. This systematic review aims to synthesize thecurrent understanding of breast angiosarcoma, covering its classification, epidemiology, clinical features,diagnostic approaches, treatment modalities, prognostic indicators, and future research directions, to providea comprehensive overview for clinicians and researchers.