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Breast sarcoma: Epidemiology, risk factors, clinical presentation, diagnosis, and staging

Rashmi Chugh, MD
Michael S Sabel, MD
Mary Feng, MD
Section Editors
Robert Maki, MD, PhD
Daniel F Hayes, MD
Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
Thomas F DeLaney, MD
Deputy Editors
Sadhna R Vora, MD
Wenliang Chen, MD, PhD


Breast sarcomas are rare, histologically heterogeneous nonepithelial malignancies that arise from the connective tissue within the breast [1]. They can develop de novo (primary), after radiation therapy (RT), or in the setting of lymphedema of the arm or breast after treatment of another malignancy (therapy related, secondary) [2-6]. Although the clinical features of breast sarcoma mimic those of breast carcinoma in some ways, therapy and prognosis differ dramatically.

This topic review will cover the epidemiology, risk factors, clinical features, diagnosis and staging of breast sarcoma. Treatment and prognosis are discussed elsewhere as are other nonepithelial breast tumors including breast lymphomas, phyllodes tumors (including cystosarcoma phyllodes), and desmoid tumors of the breast. (See "Breast sarcoma: Treatment" and "Breast lymphoma" and "Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy", section on 'Breast desmoids'.)


Breast sarcomas are rare; they account for less than 1 percent of all breast malignancies [7] and <5 percent of all soft tissue sarcomas [8]. In data compiled from the Surveillance, Epidemiology and End Results (SEER) Program of the National Cancer Institute, the annual incidence of breast sarcomas was 4.6 cases per million women [9].

The specific incidence of secondary, therapy-related breast sarcomas is difficult to discern. Secondary or treatment-related breast sarcomas most frequently arise following breast cancer treatment with RT. However, the absolute magnitude of the risk of a secondary breast sarcoma in women who undergo RT for breast cancer appears to be small (table 1).

The specific sarcoma subtype that is associated most strongly with treatment for a previous breast cancer is angiosarcoma. In a population-based study in Los Angeles County, the adjusted relative risk of developing an angiosarcoma for women with a prior diagnosis of breast cancer compared with women without breast cancer was 59 (95% CI 22-153) [10]. Furthermore, women who receive RT as a component of breast cancer treatment have a 9- to 16-fold increase in the relative risk of developing an angiosarcoma relative to those treated with other modalities [6,11]. However, despite these high relative risks, the absolute magnitude of risk for a treatment-related angiosarcoma after RT remains small (table 1). The association between ionizing RT and breast sarcomas is discussed in more detail below. (See 'Ionizing radiation' below.)

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Literature review current through: Nov 2017. | This topic last updated: Aug 28, 2017.
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