Recurrent Primary Angiosarcoma of the Breast: Challenges and Pitfalls in a Case Report

AIM: Primary breast angiosarcoma (PBA) is an exceptionally rare and highly aggressive vascular malignancy with poor prognosis, high recurrence rates, and limited evidence guiding management. We report a case of low- to intermediate-grade PBA characterized by multiple local recurrences and systemic progression but unusually prolonged survival, contributing valuable long-term follow-up data to the literature. CASE PRESENTATION: The patient presented with a breast lesion and cutaneous involvement. Diagnosis was confirmed by histopathological and immunohistochemical findings. The patient underwent skin-reducing mastectomy, followed by radiotherapy and multiple lines of chemotherapy after early recurrence. RESULTS: Despite progressive disease, overall survival reached 102 months from diagnosis, exceeding survival outcomes commonly reported for PBA. CONCLUSIONS: This case highlights the aggressive and refractory behavior of PBA, the importance of accurate histopathological diagnosis and multidisciplinary management, and the need for prolonged surveillance because of the high risk of recurrence. Furthermore, the unusually long survival observed in this patient highlights the complexity of disease biology and the potential value of individualized multimodal management strategies, while the contribution of specific treatments to survival remains uncertain.