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Malignant phyllodes tumors are biologically similar to primary breast sarcomas, whereas desmoid tumors are technically benign but often locally aggressive. Patients with breast sarcomas often present with a rapidly growing mass or, in cases of radiation-associated angiosarcoma, violaceous cutaneous lesions. Core needle biopsy is generally required to confirm the diagnosis of sarcomas. Staging workup includes MRI and chest imaging, although these are not required in the case of benign phyllodes or desmoid tumors. In general, localized breast sarcomas should be resected, with the extent of resection tailored to histologic subtype. Radiation and chemotherapy can be used in the neoadjuvant or adjuvant setting, but data are limited, so treatment decisions should be made on an individualized basis. Systemic therapy options for metastatic disease and refractory breast desmoids mimic those used for the same histologies when present in other sites. Given the rarity and heterogeneity of breast sarcoma, as well as limited literature describing these entities, expert multidisciplinary evaluation is crucial for optimal decision making.<\/jats:p>","DOI":"10.1200\/edbk_321341","type":"journal-article","created":{"date-parts":[[2021,5,19]],"date-time":"2021-05-19T16:08:30Z","timestamp":1621440510000},"page":"390-404","source":"Crossref","is-referenced-by-count":27,"title":["Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations"],"prefix":"10.1200","author":[{"given":"George Z.","family":"Li","sequence":"first","affiliation":[{"name":"Department of Surgery, Brigham and Women\u2019s Hospital and Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chandrajit P.","family":"Raut","sequence":"additional","affiliation":[{"name":"Department of Surgery, Brigham and Women\u2019s Hospital and Harvard Medical School, Boston, MA"},{"name":"Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly K.","family":"Hunt","sequence":"additional","affiliation":[{"name":"Department of Breast Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, University of California San Francisco, San Francisco, CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rashmi","family":"Chugh","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"233","reference":[{"key":"bibr2-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.3322\/caac.21590"},{"key":"bibr3-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjc.6601920"},{"key":"bibr4-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1016\/S0039-6109(05)70445-X"},{"key":"bibr5-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-199101000-00045"},{"key":"bibr6-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1007\/s10549-010-0915-y"},{"key":"bibr7-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1016\/S0360-3016(99)00444-7"},{"key":"bibr8-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2003.06.080"},{"key":"bibr9-EDBK_321341","first-page":"5358","volume":"48","author":"Li FP","year":"1988","journal-title":"Cancer Res"},{"key":"bibr10-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djk002"},{"key":"bibr11-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19880801)62:3<652::AID-CNCR2820620334>3.0.CO;2-4"},{"key":"bibr12-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(194805)1:1<3::AID-CNCR2820010103>3.0.CO;2-7"},{"key":"bibr13-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1080\/02841860802047387"},{"key":"bibr14-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.24462"},{"key":"bibr15-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(194805)1:1<64::AID-CNCR2820010105>3.0.CO;2-W"},{"key":"bibr16-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.1993.tb01008.x"},{"key":"bibr17-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197208)30:2<562::AID-CNCR2820300237>3.0.CO;2-V"},{"key":"bibr18-EDBK_321341","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.21531"},{"key":"bibr19-EDBK_321341","unstructured":"WHO Classification of Tumours Editorial Board. 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