Primary breast angiosarcoma is an uncommon disease; it represents 8% of breast sarcomas and 1% of all malignant breast lesions. It occurs more frequently in females (4∶1) mainly young, typically between second and fourth decades of life. Its etiology is not defined. It may present as small or large lesions. The neoplasm uses to disseminate by hematogenous, more than lymphatic pathways. Surgery is the only treatment with curative potential. Chemotherapy and radiation therapy have not obtained good responses.
Tumor size, and involvement of surrounding structures impact survival are important prognostic factors. Reported mean survival is around 20 months.
We report 5 cases of primary breast angiosarcoma. It is remarkable the occurrence in a male patient, and the long survival of two patients, with 6 and 18 years of disease free survival. Three patients were younger than 19 years old. One patient showed the typical tumor behavior, with early local recurrence and disseminated disease, followed by rupture of a hepatic metastatic seed with hemoperitoneum and death.
We describe the histopathologic features of each lesion, showing that degree of differentiation is not always related with clinical behavior.