{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T10:36:39Z","timestamp":1770546999088,"version":"3.49.0"},"reference-count":115,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T00:00:00Z","timestamp":1690934400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Breast sarcomas (BSs), phyllodes tumors (PTs), and desmoid tumors (DTs) are rare entities that arise from connective tissue. BSs can be classified as either primary or secondary, whether they develop de novo or after radiation exposure or lymphedema. PIK3CA seems to play an important common role in different BS. Malignant PTs show similar behavior to BSs, while DTs are locally aggressive but rarely metastasize. BSs usually present as unilateral, painless, rapidly growing masses with rare nodal involvement. The diagnosis should be based on magnetic resonance imaging and a core needle biopsy. Staging should comprise a chest computed tomography (CT) scan (except for benign PT and DT), while abdominal and pelvic CT scans and bone scans should be added in certain subtypes. The mainstay of treatment for localized BS is surgery, with margin goals that vary according to subtype. Radiotherapy and chemotherapy can be used as neoadjuvant or adjuvant approaches, but their use in these settings is not standard. Advanced BS should be treated with systemic therapy, consistent with recommendations for advanced soft tissue sarcomas of other topographies. Given the rarity and heterogeneity of these entities, multidisciplinary and multi-institutional collaboration and treatment at reference centers are critical.<\/jats:p>","DOI":"10.3390\/cancers15153933","type":"journal-article","created":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T10:57:33Z","timestamp":1690973853000},"page":"3933","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Turning the Magnifying Glass on Rare and Aggressive Entities"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6858-7341","authenticated-orcid":false,"given":"Miguel","family":"Esperan\u00e7a-Martins","sequence":"first","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Luis Costa Lab, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8556-6657","authenticated-orcid":false,"given":"Cec\u00edlia","family":"Melo-Alvim","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Sara","family":"D\u00e2maso","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8976-3199","authenticated-orcid":false,"given":"Raquel","family":"Lopes-Br\u00e1s","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"}]},{"given":"T\u00e2nia","family":"Peniche","sequence":"additional","affiliation":[{"name":"Luis Costa Lab, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1127-052X","authenticated-orcid":false,"given":"Gon\u00e7alo","family":"Nogueira-Costa","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Catarina","family":"Abreu","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Helena","family":"Luna Pais","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Rita Teixeira","family":"de Sousa","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Sofia","family":"Torres","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"}]},{"given":"Lina Marcela","family":"Gallego-Paez","sequence":"additional","affiliation":[{"name":"Luis Costa Lab, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0429-9380","authenticated-orcid":false,"given":"Marta","family":"Martins","sequence":"additional","affiliation":[{"name":"Luis Costa Lab, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"given":"Leonor","family":"Ribeiro","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4782-7318","authenticated-orcid":false,"given":"Lu\u00eds","family":"Costa","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Centro Hospitalar Universit\u00e1rio Lisboa Norte, 1649-028 Lisboa, Portugal"},{"name":"Luis Costa Lab, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"},{"name":"Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal"}]}],"member":"1968","published-online":{"date-parts":[[2023,8,2]]},"reference":[{"key":"ref_1","unstructured":"Karlin, N.J., and Wong, D.A. (2018). The Breast, Elsevier."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1200\/EDBK_321341","article-title":"Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations","volume":"41","author":"Li","year":"2021","journal-title":"Am. Soc. Clin. Oncol. Educ. Book"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1038\/sj.bjc.6601920","article-title":"Primary breast sarcoma: Clinicopathologic series from the Mayo Clinic and review of the literature","volume":"91","author":"Adem","year":"2004","journal-title":"Br. J. Cancer"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2583","DOI":"10.1200\/JCO.2003.06.080","article-title":"Prognostic Factors in Primary Breast Sarcomas: A Series of Patients with Long-Term Follow-Up","volume":"21","author":"Zelek","year":"2003","journal-title":"J. Clin. Oncol."},{"key":"ref_5","unstructured":"Bassett, L.W., Mahoney, M.C., Apple, S., and D\u2019Orsi, C. (2011). Breast Imaging Expert Radiology, ELSEVIER Saunders."},{"key":"ref_6","first-page":"4056459","article-title":"Stewart-Treves Syndrome Involving Chronic Lymphedema after Mastectomy of Breast Cancer","volume":"2017","author":"Mesli","year":"2017","journal-title":"Case Rep. Surg."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1016\/S1470-2045(22)00561-7","article-title":"Treatment-related thoracic soft tissue sarcomas in US breast cancer survivors: A retrospective cohort study","volume":"23","author":"Veiga","year":"2022","journal-title":"Lancet Oncol."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"S\u00e1nchez-Heras, A.B., Cajal, T.R.Y., Pineda, M., Aguirre, E., Gra\u00f1a, B., Chirivella, I., Balma\u00f1a, J., and Brunet, J. (2023). SEOM Clinical Guideline on Heritable TP53-Related Cancer Syndrome (2022). Clin. Transl. Oncol., 1\u20137.","DOI":"10.1007\/s12094-023-03202-9"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.6004\/jnccn.2018.7081","article-title":"Screening Mammography for Average-Risk Women: The Controversy and NCCN\u2019s Position","volume":"16","author":"Helvie","year":"2018","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_10","unstructured":"(2023, July 20). Screening Ages and Frequencies|Cancer Screening, Diagnosis and Care. Available online: https:\/\/healthcare-quality.jrc.ec.europa.eu\/ecibc\/european-breast-cancer-guidelines\/screening-ages-and-frequencies."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"pl1","DOI":"10.1126\/scisignal.2004088","article-title":"Integrative Analysis of Complex Cancer Genomics and Clinical Profiles Using the cBioPortal","volume":"6","author":"Gao","year":"2013","journal-title":"Sci. Signal."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1158\/2159-8290.CD-12-0095","article-title":"The cBio cancer genomics portal: An open platform for exploring multidimensional cancer genomics data","volume":"2","author":"Cerami","year":"2012","journal-title":"Cancer Discov."},{"key":"ref_13","unstructured":"(2023, June 23). Angiosarcoma Project. Available online: https:\/\/ascproject.org\/."},{"key":"ref_14","unstructured":"(2023, June 23). CBioPortal for Cancer Genomics. Available online: https:\/\/www.cbioportal.org\/study\/summary?id=angs_painter_2020."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/s41591-019-0749-z","article-title":"The Angiosarcoma Project: Enabling genomic and clinical discoveries in a rare cancer through patient-partnered research","volume":"26","author":"Painter","year":"2020","journal-title":"Nat. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1038\/s41467-022-30453-x","article-title":"Clinical sequencing of soft tissue and bone sarcomas delineates diverse genomic landscapes and potential therapeutic targets","volume":"13","author":"Nacev","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_17","unstructured":"RStudio Team (2020). RStudio: Integrated Development for R, RStudio, PBC. Available online: http:\/\/www.rstudio.com\/."},{"key":"ref_18","unstructured":"Wickham, H., Fran\u00e7ois, R., Henry, L., M\u00fcller, K., and Vaughan, D. (2023, June 29). Dplyr: A Grammar of Data Manipulation. Available online: https:\/\/github.com\/tidyverse\/dplyr."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1101\/gr.239244.118","article-title":"Maftools: Efficient and comprehensive analysis of somatic variants in cancer","volume":"28","author":"Mayakonda","year":"2018","journal-title":"Genome Res."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1007\/s12032-022-01695-y","article-title":"Cell line models for drug discovery in PIK3CA-mutated colorectal cancers","volume":"39","author":"Voutsadakis","year":"2022","journal-title":"Med. Oncol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"100653","DOI":"10.1016\/j.jbior.2019.100653","article-title":"NSAID therapy for PIK3CA-Altered colorectal, breast, and head and neck cancer","volume":"75","author":"Cai","year":"2020","journal-title":"Adv. Biol. Regul."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1016\/j.bcp.2013.05.006","article-title":"MicroRNA-15b contributes to ginsenoside-Rg1-induced angiogenesis through increased expression of VEGFR-2","volume":"86","author":"Chan","year":"2013","journal-title":"Biochem. Pharmacol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.mvr.2016.12.011","article-title":"Cryptotanshinone inhibits VEGF-induced angiogenesis by targeting the VEGFR2 signaling pathway","volume":"111","author":"Xu","year":"2017","journal-title":"Microvasc. Res."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1858","DOI":"10.1093\/hmg\/ddl108","article-title":"Neurofibroma-associated growth factors activate a distinct signaling network to alter the function of neurofibromin-deficient endothelial cells","volume":"15","author":"Munchhof","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1889","DOI":"10.1128\/MCB.01506-06","article-title":"Knockdown of ALR (MLL2) Reveals ALR Target Genes and Leads to Alterations in Cell Adhesion and Growth","volume":"27","author":"Issaeva","year":"2007","journal-title":"Mol. Cell. Biol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1113","DOI":"10.1016\/j.cell.2019.01.002","article-title":"Pharmacological Targeting of STK19 Inhibits Oncogenic NRAS-Driven Melanomagenesis","volume":"176","author":"Yin","year":"2019","journal-title":"Cell"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/j.bbrc.2010.05.103","article-title":"Phospholipase C-\u03b31 is required for the epidermal growth factor receptor-induced squamous cell carcinoma cell mitogenesis","volume":"397","author":"Xie","year":"2010","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1038\/ng.2921","article-title":"Recurrent PTPRB and PLCG1 mutations in angiosarcoma","volume":"46","author":"Behjati","year":"2014","journal-title":"Nat. Genet."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Yoo, H., La, H., Lee, E.J., Choi, H.-J., Oh, J., Thang, N.X., and Hong, K. (2020). ATP-Dependent Chromatin Remodeler CHD9 Controls the Proliferation of Embryonic Stem Cells in a Cell Culture Condition-Dependent Manner. Biology, 9.","DOI":"10.3390\/biology9120428"},{"key":"ref_30","first-page":"272","article-title":"Pleomorphic Sarcoma of Breast: A Report of Two Cases and Review of Literature","volume":"55","author":"Bansal","year":"2017","journal-title":"Acta Medica Iran."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Kokkali, S., Moreno, J.D., Klijanienko, J., and Theocharis, S. (2022). Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease?. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23084125"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"174","DOI":"10.5114\/pjp.2019.90393","article-title":"Clinicopathologic characteristics of mixed epithelial\/mesenchymal metaplastic breast carcinoma (carcinosarcoma): A meta-analysis of Chinese patients","volume":"70","author":"Xu","year":"2019","journal-title":"Pol. J. Pathol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"8828158","DOI":"10.1155\/2021\/8828158","article-title":"Sarcoma of the Breast: Clinical Characteristics and Outcomes of 991 Patients from the National Cancer Database","volume":"2021","author":"Lee","year":"2021","journal-title":"Sarcoma"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1007\/s00432-022-04000-6","article-title":"Undifferentiated pleomorphic sarcoma of the breast with neoplastic fever: Case report and genomic characterization","volume":"149","author":"Gambichler","year":"2023","journal-title":"J. Cancer Res. Clin. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Radu, I., Scripcariu, V., Panu\u021ba, A., Rusu, A., Afr\u0103s\u00e2nie, V.-A., Cojocaru, E., Ani\u021bei, M.G., Alexa-Stratulat, T., Terinte, C., and \u0218erban, C.F. (2023). Breast Sarcomas\u2014How Different Are They from Breast Carcinomas? Clinical, Pathological, Imaging and Treatment Insights. Diagnostics, 13.","DOI":"10.3390\/diagnostics13081370"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1007\/s10549-019-05432-4","article-title":"Primary and secondary breast angiosarcoma: Single center report and a meta-analysis","volume":"178","author":"Abdou","year":"2019","journal-title":"Breast Cancer Res. Treat."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"70","DOI":"10.11604\/pamj.2022.42.70.31484","article-title":"Concomitant primary angiosarcoma and invasive carcinoma of the breast: A case report","volume":"42","author":"Zghal","year":"2022","journal-title":"Pan Afr. Med. J."},{"key":"ref_38","first-page":"1299","article-title":"Primary Highly Differentiated Breast Angiosarcoma in an Adolescent Girl","volume":"26","author":"Meng","year":"2022","journal-title":"Eur. Rev. Med. Pharmacol. Sci."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/S0360-3016(99)00444-7","article-title":"An analysis of 78 breast sarcoma patients without distant metastases at presentation","volume":"46","author":"McGowan","year":"2000","journal-title":"Int. J. Radiat. Oncol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1002\/1097-0142(19900901)66:5<941::AID-CNCR2820660522>3.0.CO;2-B","article-title":"Breast Sarcoma. A Clinicopathologic Review of 25 Cases","volume":"66","author":"Pollard","year":"1990","journal-title":"Cancer"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1148\/radiology.183.3.1584913","article-title":"Angiosarcoma of the breast","volume":"183","author":"Liberman","year":"1992","journal-title":"Radiology"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"W386","DOI":"10.2214\/AJR.11.7341","article-title":"Imaging Features of Primary Breast Sarcoma","volume":"198","author":"Smith","year":"2012","journal-title":"Am. J. Roentgenol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1177\/000313480206800804","article-title":"Surgical management of primary breast sarcoma","volume":"68","author":"Shabahang","year":"2002","journal-title":"Am. Surg."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1148\/radiol.2423060163","article-title":"Mammary Angiosarcomas: Imaging Findings in 24 Patients","volume":"242","author":"Yang","year":"2007","journal-title":"Radiology"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"993","DOI":"10.2214\/ajr.158.5.1314479","article-title":"Fibrosarcoma of the breast: Mammographic findings in five cases","volume":"158","author":"Elson","year":"1992","journal-title":"Am. J. Roentgenol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/0002-9610(94)90238-0","article-title":"Sarcoma and cystosarcoma phyllodes tumors of the breast\u2014A retrospective review of 58 cases","volume":"167","author":"McGregor","year":"1994","journal-title":"Am. J. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.breast.2006.05.004","article-title":"Difficulties in the pre-operative diagnosis of phyllodes tumours of the breast: A study of 84 cases","volume":"16","author":"Foxcroft","year":"2007","journal-title":"Breast"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1136\/jclinpath-2015-203545","article-title":"Sarcoma of the breast: An update on a rare entity","volume":"69","author":"Lim","year":"2016","journal-title":"J. Clin. Pathol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"e753","DOI":"10.1016\/j.clbc.2022.07.006","article-title":"Primary and Secondary Breast Sarcoma: Clinical and Pathological Characteristics, Prognostic Factors, and Nomograms for Predicting Survival","volume":"22","author":"Osman","year":"2022","journal-title":"Clin. Breast Cancer"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1016\/j.breast.2012.01.001","article-title":"Angiosarcoma of the breast: A difficult surgical challenge","volume":"21","author":"Hui","year":"2012","journal-title":"Breast"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1002\/jso.21497","article-title":"Primary and secondary angiosarcoma of the breast: The Mayo Clinic experience","volume":"101","author":"Scow","year":"2010","journal-title":"J. Surg. Oncol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"31497","DOI":"10.1038\/srep31497","article-title":"Primary female breast sarcoma: Clinicopathological features, treatment and prognosis","volume":"6","author":"Yin","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1348","DOI":"10.1016\/j.annonc.2021.07.006","article-title":"Soft tissue and visceral sarcomas: ESMO\u2013EURACAN\u2013GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up","volume":"32","author":"Gronchi","year":"2021","journal-title":"Ann. Oncol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/0959-8049(95)00520-X","article-title":"The importance of a multidisciplinary group in the treatment of soft tissue sarcomas","volume":"32","author":"Wiklund","year":"1996","journal-title":"Eur. J. Cancer"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1002\/jso.20110","article-title":"Primary sarcoma of the breast","volume":"87","author":"Pandey","year":"2004","journal-title":"J. Surg. Oncol."},{"key":"ref_56","first-page":"1059","article-title":"Sarcoma of the breast: Implications of the extent of local therapy","volume":"64","author":"North","year":"1998","journal-title":"Am. Surg."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1097\/COC.0b013e31804b40f4","article-title":"National Surgical Patterns of Care: Regional Lymphadenectomy of Breast Sarcomas","volume":"30","author":"Gullett","year":"2007","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/S0002-9610(03)00269-1","article-title":"Primary nonphylloides breast sarcomas","volume":"186","author":"Blanchard","year":"2003","journal-title":"Am. J. Surg."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/S0039-6109(05)70445-X","article-title":"BREAST SARCOMA","volume":"76","author":"Moore","year":"1996","journal-title":"Surg. Clin. N. Am."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/S0167-8140(99)00070-5","article-title":"Role of radiotherapy in sarcoma of the breast\u2014A retrospective review of the M.D. Anderson experience","volume":"52","author":"Barrow","year":"1999","journal-title":"Radiother. Oncol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/0360-3016(93)90395-C","article-title":"Primary soft tissue sarcomas of the breast: Local-regional control with post-operative radiotherapy","volume":"27","author":"Johnstone","year":"1993","journal-title":"Int. J. Radiat. Oncol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1097\/00130404-200505000-00011","article-title":"A 14-Year Retrospective Review of Angiosarcoma","volume":"11","author":"Fury","year":"2005","journal-title":"Cancer J."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/j.amjsurg.2010.12.011","article-title":"Surgical management of radiation-induced angiosarcoma after breast conservation therapy","volume":"203","author":"Jallali","year":"2012","journal-title":"Am. J. Surg."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"2700","DOI":"10.1245\/s10434-012-2310-x","article-title":"Radiation-Associated Angiosarcoma After Breast Cancer: High Recurrence Rate and Poor Survival Despite Surgical Treatment with R0 Resection","volume":"19","author":"Seinen","year":"2012","journal-title":"Ann. Surg. Oncol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"3272","DOI":"10.3390\/curroncol29050267","article-title":"Primary Angiosarcoma of the Breast: A Single-Center Retrospective Study in Korea","volume":"29","author":"Kim","year":"2022","journal-title":"Curr. Oncol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.ejso.2013.07.088","article-title":"Role of radiation therapy in the conservative management of sarcoma within an irradiated field","volume":"40","author":"Modesto","year":"2014","journal-title":"Eur. J. Surg. Oncol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1007\/s10549-019-05172-5","article-title":"Extent of resection and role of adjuvant treatment in resected localized breast angiosarcoma","volume":"175","author":"McClelland","year":"2019","journal-title":"Breast Cancer Res. Treat."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1016\/S1470-2045(10)70023-1","article-title":"Angiosarcoma","volume":"11","author":"Young","year":"2010","journal-title":"Lancet Oncol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1007\/s11864-018-0531-3","article-title":"Current and Future Directions for Angiosarcoma Therapy","volume":"19","author":"Florou","year":"2018","journal-title":"Curr. Treat. Options Oncol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"3178","DOI":"10.1016\/j.ejca.2014.10.004","article-title":"First-line anthracycline-based chemotherapy for angiosarcoma and other soft tissue sarcoma subtypes: Pooled analysis of eleven European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group trials","volume":"50","author":"Young","year":"2014","journal-title":"Eur. J. Cancer"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1002\/cncr.22784","article-title":"Primary angiosarcomas of the breast","volume":"110","author":"Sher","year":"2007","journal-title":"Cancer"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"5269","DOI":"10.1200\/JCO.2008.17.3146","article-title":"Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma: The ANGIOTAX Study","volume":"26","author":"Penel","year":"2008","journal-title":"J. Clin. Oncol."},{"key":"ref_73","first-page":"2303","article-title":"Angiosarcoma: A review of diagnosis and current treatment","volume":"9","author":"Cao","year":"2019","journal-title":"Am. J. Cancer Res."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1016\/S0140-6736(12)60651-5","article-title":"Pazopanib for metastatic soft-tissue sarcoma (PALETTE): A randomised, double-blind, placebo-controlled phase 3 trial","volume":"379","author":"Blay","year":"2012","journal-title":"Lancet"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"499","DOI":"10.6004\/jnccn.2016.0058","article-title":"Antitumor Response of VEGFR2- and VEGFR3-Amplified Angiosarcoma to Pazopanib","volume":"14","author":"Ravi","year":"2016","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1080\/0284186X.2016.1234068","article-title":"Pazopanib in advanced vascular sarcomas: An EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis","volume":"56","author":"Jones","year":"2017","journal-title":"Acta Oncol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"3133","DOI":"10.1200\/JCO.2008.20.4495","article-title":"Phase II Study of Sorafenib in Patients with Metastatic or Recurrent Sarcomas","volume":"27","author":"Maki","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1007\/s11523-013-0299-0","article-title":"Low level of baseline circulating VEGF-A is associated with better outcome in patients with vascular sarcomas receiving sorafenib: An ancillary study from a phase II trial","volume":"9","author":"Penel","year":"2014","journal-title":"Target. Oncol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"e6275","DOI":"10.1002\/ccr3.6275","article-title":"Primary dedifferentiated liposarcoma of the breast: A case report","volume":"10","author":"Shrestha","year":"2022","journal-title":"Clin. Case Rep."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1016\/S0046-8177(86)80640-2","article-title":"Liposarcoma of the breast: A clinicopathologic study of 20 cases","volume":"17","author":"Austin","year":"1986","journal-title":"Hum. Pathol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"196","DOI":"10.9738\/INTSURG-D-15-00183.1","article-title":"Breast Leiomyosarcoma: A Systematic Review and Recommendations for Management","volume":"104","author":"Ilyas","year":"2019","journal-title":"Int. Surg."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"102495","DOI":"10.1016\/j.amsu.2021.102495","article-title":"Leiomyosarcoma of the male breast: Case report","volume":"67","author":"Cheikh","year":"2021","journal-title":"Ann. Med. Surg."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/j.breast.2011.03.001","article-title":"Leiomyosarcoma of the breast: A case report and review of the literature about therapeutic management","volume":"20","author":"Fujita","year":"2011","journal-title":"Breast"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1200\/JOP.19.00476","article-title":"Primary Alveolar Rhabdomyosarcoma of the Breast in a 17-Year-Old Girl","volume":"16","author":"Mance","year":"2020","journal-title":"JCO Oncol. Pract."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1159\/000487750","article-title":"Primary Rhabdomyosarcoma of the Breast: Imaging Findings and Literature Review","volume":"13","author":"Bayramoglu","year":"2018","journal-title":"Breast Care"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1002\/(SICI)1096-911X(199709)29:3<181::AID-MPO4>3.0.CO;2-9","article-title":"Primary and Metastatic Rhabdomyosarcoma in the Breast: Neoplasms of Adolescent Females, a Report from the Intergroup Rhabdomyosarcoma Study","volume":"29","author":"Hays","year":"1997","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/j.ejro.2017.11.001","article-title":"Primary breast chondrosarcoma: Imaging and pathological findings","volume":"4","author":"Amadu","year":"2017","journal-title":"Eur. J. Radiol. Open"},{"key":"ref_88","unstructured":"Limaiem, F., and Kashyap, S. (2023). StatPearls, StatPearls Publishing."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"665","DOI":"10.5858\/arpa.2016-0042-RA","article-title":"Phyllodes Tumor of the Breast: Histopathologic Features, Differential Diagnosis, and Molecular\/Genetic Updates","volume":"140","author":"Zhang","year":"2016","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_90","doi-asserted-by":"crossref","unstructured":"Chao, X., Chen, K., Zeng, J., Bi, Z., Guo, M., Chen, Y., Yao, Y., Wu, W., Liang, S., and Nie, Y. (2019). Adjuvant radiotherapy and chemotherapy for patients with breast phyllodes tumors: A systematic review and meta-analysis. BMC Cancer, 19.","DOI":"10.1186\/s12885-019-5585-5"},{"key":"ref_91","first-page":"761","article-title":"Phyllodes Tumour of the Male Breast","volume":"11","author":"Bielsen","year":"1987","journal-title":"Histopathology"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"361469","DOI":"10.1155\/2013\/361469","article-title":"Phyllodes Tumor of Breast: A Review Article","volume":"2013","author":"Mishra","year":"2013","journal-title":"ISRN Surg."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.breast.2018.11.009","article-title":"Phyllodes tumors of the breast. The treatment results for 340 patients from a single cancer centre","volume":"43","author":"Mitus","year":"2019","journal-title":"Breast"},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.47162\/RJME.62.4.16","article-title":"Diagnostic difficulties in giant benign phyllodes tumor","volume":"62","author":"Matei","year":"2022","journal-title":"Rom. J. Morphol. Embryol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1038\/modpathol.3800756","article-title":"Phyllodes tumors of the breast segregate in two groups according to genetic criteria","volume":"20","author":"Savignoni","year":"2007","journal-title":"Mod. Pathol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/j.prp.2006.05.006","article-title":"Correlation of p53 and MIB-1 expression with both the systemic recurrence and survival in cases of phyllodes tumors of the breast","volume":"202","author":"Yonemori","year":"2006","journal-title":"Pathol.-Res. Pract."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1111\/his.12329","article-title":"Immunohistochemical expression of homeoproteins Six1 and Pax3 in breast phyllodes tumours correlates with histological grade and clinical outcome","volume":"64","author":"Tan","year":"2014","journal-title":"Histopathology"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1309\/D206-DLF8-WDNC-XJ8K","article-title":"Hormonal Receptors Expression in Epithelial Cells of Mammary Phyllodes Tumors Correlates with Pathologic Grade of the Tumor","volume":"118","author":"Tse","year":"2002","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_99","unstructured":"(2023, July 20). Desmoid Tumor-Symptoms, Causes, Treatment|NORD. Available online: https:\/\/rarediseases.org\/rare-diseases\/desmoid-tumor\/."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1159\/000507842","article-title":"Desmoid Type Fibromatosis of the Breast: Ten-Year Institutional Results of Imaging, Histopathology, and Surgery","volume":"16","author":"Lorenzen","year":"2020","journal-title":"Breast Care"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1038\/s41698-022-00308-1","article-title":"Molecular pathogenesis of desmoid tumor and the role of \u03b3-secretase inhibition","volume":"6","author":"Federman","year":"2022","journal-title":"NPJ Precis. Oncol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"1832","DOI":"10.21037\/gs-22-271","article-title":"Bilateral breast desmoid-type fibromatosis, case report and literature review","volume":"11","author":"Hennuy","year":"2022","journal-title":"Gland. Surg."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"2417","DOI":"10.1056\/NEJMoa1805052","article-title":"Sorafenib for Advanced and Refractory Desmoid Tumors","volume":"379","author":"Gounder","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.ejca.2017.02.001","article-title":"Imatinib induces sustained progression arrest in RECIST progressive desmoid tumours: Final results of a phase II study of the German Interdisciplinary Sarcoma Group (GISG)","volume":"76","author":"Kasper","year":"2017","journal-title":"Eur. J. Cancer"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1016\/S1470-2045(19)30276-1","article-title":"Pazopanib or methotrexate\u2013vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): A non-comparative, randomised, open-label, multicentre, phase 2 study","volume":"20","author":"Toulmonde","year":"2019","journal-title":"Lancet Oncol."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1056\/NEJMoa2210140","article-title":"Nirogacestat, a \u03b3-Secretase Inhibitor for Desmoid Tumors","volume":"388","author":"Gounder","year":"2023","journal-title":"N. Engl. J. Med."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"S1227","DOI":"10.1016\/j.annonc.2022.07.1591","article-title":"1488MO Initial results of phase II\/III trial of AL102 for treatment of desmoid tumors (DT)","volume":"33","author":"Gounder","year":"2022","journal-title":"Ann. Oncol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1007\/s10549-019-05245-5","article-title":"Breast desmoid tumor management in France: Toward a new strategy","volume":"176","author":"Lusque","year":"2019","journal-title":"Breast Cancer Res. Treat."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/1477-7819-4-32","article-title":"Management of a patient with multiple recurrences of fibromatosis (desmoid tumor) of the breast involving the chest wall musculature","volume":"4","author":"Povoski","year":"2006","journal-title":"World J. Surg. Oncol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"184","DOI":"10.11604\/pamj.2022.41.184.28549","article-title":"Fibromatose desmo\u00efde mammaire: \u00c0 propos de deux cas et revue de la litt\u00e9rature","volume":"41","author":"Laakom","year":"2022","journal-title":"Pan Afr. Med. J."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1245\/s10434-007-9580-8","article-title":"Desmoid Tumors (Fibromatoses) of the Breast: A 25-Year Experience","volume":"15","author":"Neuman","year":"2007","journal-title":"Ann. Surg. Oncol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"3553","DOI":"10.1200\/JCO.2010.33.5489","article-title":"Prognostic Factors Influencing Progression-Free Survival Determined from a Series of Sporadic Desmoid Tumors: A Wait-and-See Policy According to Tumor Presentation","volume":"29","author":"Salas","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"4028","DOI":"10.1245\/s10434-012-2638-2","article-title":"Desmoid Tumor: Analysis of Prognostic Factors and Outcomes in a Surgical Series","volume":"19","author":"Mullen","year":"2012","journal-title":"Ann. Surg. Oncol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1200\/JCO.1999.17.1.158","article-title":"Desmoid Tumor: Prognostic Factors and Outcome After Surgery, Radiation Therapy, or Combined Surgery and Radiation Therapy","volume":"17","author":"Ballo","year":"1999","journal-title":"J. Clin. Oncol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"2045","DOI":"10.1002\/(SICI)1097-0142(19991115)86:10<2045::AID-CNCR23>3.0.CO;2-F","article-title":"Extremity and trunk desmoid tumors: A multifactorial analysis of outcome","volume":"86","author":"Merchant","year":"1999","journal-title":"Cancer"}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/15\/15\/3933\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:24:25Z","timestamp":1760127865000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/15\/15\/3933"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8,2]]},"references-count":115,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2023,8]]}},"alternative-id":["cancers15153933"],"URL":"https:\/\/doi.org\/10.3390\/cancers15153933","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,8,2]]}}}