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Breast sarcomas are a very rare form of breast cancer, accounting for fewer than 1% of all breast cancers. Unlike more common kinds of breast cancer which begin in the milk ducts, breast sarcomas begin in the connective tissue that supports the ducts and lobules of the breast.
Breast sarcomas can be primary or secondary tumors. Some kinds of inherited genetic disorders are known to increase one’s risk of developing a primary breast sarcoma. Just as with other forms of breast cancer, however, the reason for the development of a primary breast sarcoma is often unknown. Secondary tumors can develop after radiation therapy, chronic lymphedema, certain chemicals, some forms of chemotherapy, and immune system diseases.
Because breast sarcoma cells are more like the connective tissue in the breast than the ductal breast tissue, they act differently than more common kinds of breast cancer. They are often high grade, which means that the cells are very abnormal-looking or dividing rapidly. Breast sarcomas also tend to be larger at diagnosis than other types of breast tumors.
Staging and treatment of breast sarcomas differ from other types of breast cancer. Lymph node status is not as important in staging breast sarcomas as in other kinds of breast cancer. When sarcomas spread, they typically do not travel through the lymphatic system. Even in the case of large breast sarcomas, the lymph nodes are usually negative for cancer, and axillary node dissection (removal of the underarm lymph nodes) is usually not required.
Local therapy is aimed at preventing the cancer from coming back in the breast, and will include surgery such as wide excision, in which the tumor and extra tissue are removed, or mastectomy, in which the whole breast is removed. Radiation therapy may also be given.
Systemic therapy is used to prevent the cancer from coming back or from spreading to another part of the body. Systemic therapy for a sarcoma may be recommended if the tumor is very large or is known to have spread outside of the breast, and includes chemotherapy. Because sarcoma tumor cells are not ductal breast cells, they do not typically have hormone or HER2 receptors, so endocrine and HER2- targeted therapies are generally not used. Your oncology team will recommend a treatment plan based on what is known about breast sarcomas in general and tailored specifically to your disease.