Topic Completed: 1 June 2016

Minor changes: 21 June 2020

Revised: 28 January 2019

Copyright: (c) 2002-2019,, Inc.

PubMed Search: Breast sarcoma [title]

Emily S. Reisenbichler, M.D.
Page views in 2019: 1,606
Page views in 2020 to date: 929
Cite this page: Reisenbichler ES General. website. Accessed August 13th, 2020.
Essential features
  • Rare, less than 1% of malignant breast tumors
  • Represent < 5% of all sarcomas
  • Treatment and behavior (metastases) are more like soft tissue sarcoma than like breast carcinoma
  • May arise as primary tumors or secondary to radiation or chronic lymphedema
  • Most common subtype is angiosarcoma
  • Primary breast sarcoma may be associated with genetic conditions such as Li-Fraumeni syndrome and neurofibromatosis type 1 (Surg Clin North Am 2008;88:451)
  • Also secondary to radiation for breast carcinoma or other thoracic malignancy with an incidence of 0.03 - 0.2% and average latency period of 10 years (Cases J 2008;1:313)
Clinical features
  • Most arise as a unilateral, rapidly enlarging palpable mass (Expert Rev Anticancer Ther 2014;14:705)
  • Median age of diagnosis 49.5 years, although tendency to occur in younger age when primary sarcoma compared to sarcoma secondary to radiation (J Clin Pathol 2016;69:373)
  • Lungs and liver are most common sites of metastases
Radiology description
Prognostic factors
  • Favorable prognostic factors: non-menopausal status, no residual tumor following surgical resection (R0), non angiosarcoma subtype, grade 1 - 2 histology (Radiother Oncol 2007;85:355)
  • Size < 5 cm has been shown to be the most favorable prognostic indicator of overall survival (Am J Surg 2008;196:559)
  • Previous irradiation associated with poorer prognosis (Eur J Surg Oncol 2011;37:703)
  • Angiosarcoma is the worst subtype
Gross description
  • Generally large, median size of 5.25 cm
  • Blue / purple skin discoloration may be seen in angiosarcoma
Microscopic (histologic) description
  • Dependent on sarcoma subtype
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