Definition
A tumor composed of malignant cells with endothelial differentiation.
Clinical Features
Incidence
Angiosarcoma (AS) of the breast is a rare disease accounting for about 1% of all soft tissue breast tumors.
It may present as primary AS, without previous history of mammary carcinoma or any associated factor, or as secondary AS, most commonly associated with previous breast irradiation.
Primary AS represents <0.05% of all primary breast cancers.
Secondary AS presents a median of 10.5 years after radiotherapy for breast cancer. In those patients undergoing breast conserving surgery with adjuvant radiotherapy, the estimated incidence of radiation-induced AS is 0.05–0.3%.
AS rarely occurs after mastectomy with axillary dissection without irradiation and during pregnancy.
Age
Primary AS more frequently affects young and middle-aged women (median age 30–55 ys).
Secondary AS presents in older women (median age 67–71 ys) after radiotherapy for breast cancer.
Sex
Rare cases of AS have...
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References and Further Reading
Bowman, E., Oprea, G., Okoli, J., Gundry, K., et al. (2012). Pseudoangiomatous stromal hyperplasia (PASH) of the breast: A series of 24 patients. Breast Journal, 18(3), 242–247.
Lucas, D. R. (2009). Angiosarcoma, radiation-associated angiosarcoma, and atypical vascular lesion. Archives of Pathology & Laboratory Medicine, 133, 1804–1809.
Mentzel, T., Schildhaus, H. U., Palmedo, G., Buttner, R., & Kutzner, H. (2012). Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy andcontrol cases: Clinicopathological, immunohistochemical and molecular analysis of 66 cases. Modern Pathology, 25, 75–85.
Nascimento, A. F., Raut, C. P., & Fletcher, C. (2008). Primary angiosarcoma of the breast, clinicopathologic analysis of 49 cases, suggesting that grade is not prognostic. The American Journal of Surgical Pathology, 32, 1896–1904.
Rosen, P. P., Kimmel, M., & Ernsberger, D. (1988). Mammary angiosarcoma, the prognostic significance of tumor differentiation. Cancer, 62, 2145–2151.
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Pisacane, A. (2020). Angiosarcoma of the Breast. In: Sapino, A., Kulka, J. (eds) Breast Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-62539-3_4699
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DOI: https://doi.org/10.1007/978-3-319-62539-3_4699
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