Primary angiosarcoma of the breast: a rare entity

Dyan D’Souza, Ann Sunny, Rahul Sima, Anthony P. Rozario


Primary angiosarcoma of the breast is a rare form of breast cancer, accounting for only 0.04% of all malignant breast tumours. We report a 35-year-old woman who presented with a recurrent lump in the left breast for four months. Fine needle aspiration cytology (FNAC) was suspicious of angiosarcoma and this diagnosis was confirmed on core needle biopsy. Modified radical mastectomy was performed as sole treatment. No chemo radiotherapy was given as tumor was grade I, margins were negative and there was no lymph node involvement. She is on regular follow up and no recurrence reported since the surgery. The 5-year disease-free survival for grade I tumours can be as high as 76%, and up to 70% for grade II tumours whereas for grade III tumours it is reportedly about 15%. Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection. Surgery is the mainstay of treatment with a limited role for chemotherapy and radiotherapy.


Angiosarcoma, Primary angiosarcoma of the breast

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Schmidt GB. Ueber das angiosarkom der Mamma. Arch Klin Chir. 1887;36:421-7.

Adem C, Reynolds C, Ingle JN. Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature. Br J Cancer. 2004;91:237-24.

Vorburger SA, Xing Y, Hunt KK. Angiosarcoma of the breast. Cancer. 2005;104(12):2682-8.

Wang XY, Jakowski J, Tawfik OW, Thomas PA, Fan F. Angiosarcoma of the breast: a clinicopathologic analysis of cases from the last 10 years. Annals Diagnostic Pathology. 2009;13(3):147-50.

Scow JS, Reynolds CA, Degnim AC, Petersen IA, Jakub JW, Boughey JC. Primary and secondary angiosarcoma of the breast: the Mayo Clinic experience. J Surgical Oncology. 2010;101(5):401-7.

Bernathova M, Jaschke W, Pechlahner C, Zelger B, Bodner G. Primary angiosarcoma of the breast associated Kasabach-Merritt syndrome during pregnancy. Breast. 2006;15(2):255-8.

Georgiannos SN, Sheaff M. Angiosarcoma of the breast: a 30 year perspective with an optimistic outlook. British J Plastic Surgery. 2003;56(2):129-34.

Ohta M, Tokuda Y, Kuge S. A case of angiosarcoma of the breast. Japanese J Clinical Oncology. 1997;27(2):91-9.

Jaywantrao SB, Yadavrao AK, Patil MV, Wader JV, Nangare N, Patil PP. Int J Surg Case Rep. 2013;4(4):362-4.

Kikawa Y, Konishi Y, Nakamoto Y, Harada T, Takeo M, Ogata M, Katsuyama E. Angiosarcoma of the breast - specific findings of MRI. Breast Cancer. 2006;13(4):369-73.

Penel N, Bui BN, Bay JO, Cupissol D, Ray-Coquard I, Neumann PS, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clinical Oncology. 2008;26(32):5269-74.

Fodor J, Orosz Z, Szabo E. Angiosarcoma after conservation treatment for breast carcinoma: our experience and a review of the literature. J Am Acad Dermatol. 2006;54:499-504.

Rosen PP, Kimmel M, Ernsberger D. Mammary angiosarcoma: the prognostic significance of tumour differentiation. Cancer. 1988;62(10):2145-51.