Abstract
Primary angiosarcoma of the breast (PAB) is an exceptionally rare and aggressive malignancy, representing only 0.05% of all breast tumors. Its low incidence and nonspecific presentation frequently lead to diagnostic delays, as current clinical guidelines remain limited. We report the case of a 27-year-old woman who presented with a rapidly enlarging inflammatory mass in the right breast, initially interpreted as a possible abscess. Lack of response to empirical treatment and continued growth prompted further evaluation. Imaging and subsequent biopsy established the diagnosis of a primary low-grade angiosarcoma. Staging studies showed no evidence of metastasis, and the patient underwent a simple right mastectomy with curative intent. This case underscores the diagnostic challenges associated with PAB, particularly its ability to mimic benign or infectious conditions and its variable imaging features, which differ according to histologic grade. Accurate diagnosis requires correlating the clinical course with imaging findings and pathological assessment. Breast MRI is considered the most informative imaging modality, but tissue biopsy remains essential for confirmation. At present, complete surgical excision with clear margins is the standard approach, although the benefit of adjuvant therapy is still debated. Given the absence of well-defined management protocols, each case requires individualized, multidisciplinary evaluation.
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Copyright (c) 2026 Sophie Caulier, Stéphane Gillerot, Marie A. Labaisse, Nathalie Renard

