Open Access Peer-reviewed Case Report

Primary angiosarcoma of the breast: A clinical case and review of the literature

Main Article Content

Lena Marinova corresponding author
Bistra G. Yordanova
Doroteya V. Malinova


We present here a rare case of primary mammary angiosarcoma in 48-year-old female patient. After 3 years without treatment, the woman presented to the hospital with locally advanced tumor in right mammary gland, involving the overlying skin and bleeding. Radical mastectomy was performed with axillary lymph-node dissection. The CT scan revealed solitary liver metastasis. After an overview of different cases of primary angiosarcoma of the breast published in the literature, we discuss the importance of histological criteria and immunohistochemical methods, as well as the optimal multimodal treatment in these patients. Poorly differentiated primary mammary angiosarcoma (grade 3) is an invasive neoplasm with high risk of local recurrence and distant metastases. The multimodal treatment involves radical mastectomy with or without axillary lymph-node dissection. Adjuvant radiotherapy and adjuvant chemotherapy help the local tumor control, reduce recurrences and increase overall survival.

primary mammary angiosarcoma, radical mastectomy, radiotherapy, chemotherapy, multimodal treatment

Article Details

How to Cite
Marinova, L., Yordanova, B., & Malinova, D. (2020). Primary angiosarcoma of the breast: A clinical case and review of the literature. Current Cancer Reports, 2(1), 28-33.


  1. Donnell RM, Rosen PP, Lieberman PH, et al. Angiosarcoma and other vascular tumors of the breast. The Americal Journal Surgical Pathology, 1981, 5: 629-642.
  2. Rosen PP, Kimmel M and Ernsberger D. Mammary angiosarcoma: the prognostic significance of tumor differentiation. Cancer, 1988, 62(10): 2145-2151.;2-O
  3. Chen KT, Kirkegaard DD and Bocian JJ. Angiosarcoma of the breast. Cancer, 1980, 46(2): 368-371.;2-E
  4. Hui A, Henderson M, Speakman D, et al. Angiosarcoma of the breast: a difficult surgical challenge. The Breast, 2012, 21(4): 584-589.
  5. May DS and Stroup NE. The incidence of sarcomas of the breast among women in the United States, 1973-1986. Plastic and Reconstructive Surgery, 1991, 87(1): 193-194.
  6. Hodgson NC, Bowen-Wells C, Moffat F, et al. Angiosarcomas of the breast: a review of 70 cases. American Journal of Clinical Oncology, 2007, 30(6): 570-573.
  7. Kaklamanos IG, Birbas K, Syrigos KN, et al. Breast angiosarcoma that is not related to radiation exposure: a comprehensive review of the literature. Surgery Today, 2011, 41: 163-168.
  8. Torres KE, Ravi V, Kin K, et al. Long-term outcomes in patients with radiation-associated angiosarcomas of the breast following surgery and radiotherapy for breast cancer. Annals Surgical Oncology, 2013, 20: 1267-1274.
  9. Vorburger SA, Xing Y, Hunt KK, et al. Angiosarcoma of the breast. Cancer, 2005, 104(12): 2682-2688.
  10. Desbiens C, Hogue JC and Lvesque Y. Primary Breast Angiosarcoma. Oncological Medicine, 2011, Article ID 517047, 5 pages.
  11. Aydogdu M and Trams G. Angiosarcoma of the breast after conservatively operated breast carcinoma - a sequelae of adjuvant radiotherapy? Geburtshilfe Frauenheilkd, 1996, 56(1): 60-62.
  12. Lim RF and Goei R. Angiosarcoma of the Breast, Radio- Graphics (Best Cases from the AFIP), 2007, 27(supple 1): S125-S130.
  13. Bhosale SJ, Kshirsagar AY, Patil MV, et al. Primary angiosarcoma of breast: A case report. International Journal of Surgery Case Reports, 2013, 4(4): 362-364.
  14. Kunkiel M, Maczkiewicz M, Jagieo-Gruszfeld A, et al. Primary angiosarcoma of the breast-series of 11 consecutive cases-a single-centre experience. Current Oncology, 2018, 25(1): 50-53.
  15. Arora TK, Terracina KP, Soong J, et al. Primary and secondary angiosarcoma of the breast. Gland Surgery, 2014, 3(1): 28-34.
  16. Wang XY, Jakowski J, Tawfik OW, et al. Angiosarcoma of the breast: a clinicopathologic analysis of cases from the last 10 years. Annals of Diagnostic Pathology, 2009, 13(3): 147-150.
  17. Scow JS, Reynolds CA, Degnim AC, et al. Primary and secondary angiosarcoma of the breast: the Mayo Clinic experience. Journal of Surgical Oncology, 2010, 101(5): 401-407.
  18. Vemulapalli S, Mita A, Alvarado Y, et al. The emerging role of mammalian target of rapamycin inhibitors in the treatment of sarcomas. Targeted Oncology, 2011, 6(1): 29-39.
  19. Iacoponi S, Calleja J, Hernandez G, et al. Primary breast angiosarcoma in a young woman. International Journal of Surgery Case Reports, 2016, 24: 101-103.
  20. Seinen JM, Styring E, Verstappen V, et al. Radiationassociated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection. Annals of Surgical Oncology, 2012, 19: 2700- 2706.
  21. Jallali N, James S, Searle A, et al. Surgical management of radiation-induced angiosarcoma after breast conservation therapy. The American Journal Surgery, 2012, 203(2): 156- 161.
  22. Morgan EA, Kozono DE, Wang Q, et al. Cutaneous radiation-associated angiosarcoma of the breast: poor prognosis in a rare secondary malignancy. Annals of Surgical Oncology volume, 2012, 19: 3801-3808.
  23. Nicolas MM, Nayar R, Yeldandi A, et al. Pulmonary metastasis of a postradiation breast epithelioid angiosarcoma mimicking adenocarcinoma.Acta Cytological, 2006, 50(6): 672-676.
  24. Johnson CM and Garguilo GA. Angiosarcoma of the breast: a case report and literature review. Current Surgery, 2002, 59(5): 490-494.
  25. Georgiannos SN and Sheaff M. Angiosarcoma of the breast: a 30 year perspective with an optimistic outlook. British Journal of Plastic Surgery, 2003, 56(2): 129-134.
  26. Ohta M, Tokuda Y and Kuge S. A case of angiosarcoma of the breast. Japanese Journal of Surgical Oncology, 1997, 27(2): 91-94.
  27. Rosen PP. Vascular tumors of the breast: III. The American Journal of Surgical Pathology, 1985, 9: 652-658.
  28. Fisher CJ, Hanby AM, Robinson L, et al. Mammary hamartoma- a review of 35 cases. Histopathology, 1992, 20(2): 99-106.
  29. Zelek L, Llombart-Cussac A and Terrier P. Prognostic factors in primary breast sarcomas: a series of patients with long-term follow-up. Journal of Clinical Oncology, 2003, 21(13): 2583-2588.
  30. Cornelis A, Verjans M, Van Den Bosch T, et al. Efficacy and safety of direct and frontal macrobiopsies in breast cancer. European Journal of Cancer Prevention, 2009, 18(4): 280- 284.
  31. Jagtap SV, Shukla D, Bonde VS, et al. Primary angiosarcoma of the breast: an uncommon histopathological subtype. Journal of Clinical and Diagnostic Research, 2015, 9(12): 5-6.
  32. Glazebrook KN, Magut MJ and Reynolds C. Angiosarcoma of the Breast. American Journal of Roentgenology, 2008, 190(2): 533-538.
  33. Hart J and Mandavilli S. Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Archives of Pathology and Laboratory Medicine, 2011, 135(2): 268-272.
  34. Folpe AL, Chand EM, Goldblum JR, et al. Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms from potential mimics.The American Journal of Surgical Pathology, 2001, 25(8): 1061-1066.
  35. Bennani A, Chbani L, Lamchahab M, et al. Primary angiosarcoma of the breast: a case report. Diagnostic Pathology, 2013, 8: 66.
  36. Sher T, Hennessy BT, Valero V, et al. Primary angiosarcomas of the breast. Cancer, 2007, 110(1): 173-178.
  37. Bousquet G, Confavreux C, Magne N, et al. Outcome and prognostic factors in breast sarcoma: a multicenter study from the rare cancer network. Radiotherapy Oncology, 2007, 85(3): 355-3561.
  38. Horisawa N, Adachi Y, Sawaki M, et al. A case of radiationassociated angiosarcoma after breast cancer. Surgical Case Reports, 2018, 4(1): 131.