Navigating the challenges of primary angiosarcoma of the breast: Comprehensive review and a case report

Primary angiosarcoma of the breast (PBA), comprising less than 1% of all malignant breast tumors, presents challenges in both diagnosis and management. Given the unclear pathogenesis, consensus on the optimal treatment for primary angiosarcoma is lacking. Surgery remains the mainstay of management, while the role of adjuvant therapies remains controversial. Here, we present a case of primary angiosarcoma in a teenager with thalassemia. Following complete surgical excision, the patient could not to complete the full course of chemotherapy due to an intolerable side effect. Nonetheless, the patient remained free of recurrence or metastasis during the 1-year follow-up period. This case underscores the importance of thorough tumor removal in the management of primary angiosarcoma, emphasizing the need for interdisciplinary collaboration to refine treatment strategies and customize optimal approaches for individual patients. We also comprehensively reviewed a large body of literature and relevant data on the epidemiology, molecular mechanisms, and clinical treatment of PBA, in order to provide a better understanding of this disease.