{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:36:40Z","timestamp":1787027800140,"version":"build-2736575974"},"reference-count":86,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2022,3,26]],"date-time":"2022-03-26T00:00:00Z","timestamp":1648252800000},"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>Primary dermal sarcomas (PDS) belong to a highly clinically, genetically and pathologically heterogeneous group of rare malignant mesenchymal tumours primarily involving the dermis or the subcutaneous tissue. The tumours are classified according to the mesenchymal tissue from which they originate: dermal connective tissue, smooth muscle or vessels. Clinically, PDS may mimic benign soft tissue lesions such as dermatofibromas, hypertrophic scarring, etc. This may cause substantial diagnostic delay. As a group, PDS most commonly comprises the following clinicopathological forms of dermal sarcomas: dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), dermal undifferentiated pleomorphic sarcoma (DUPS), leiomyosarcoma (LMS), and vascular sarcomas (Kaposi\u2019s sarcoma, primary angiosarcoma, and radiation-induced angiosarcoma). This clinical entity has a broad spectrum regarding malignant potential; however, local aggressive behaviour in some forms causes surgical challenges. Preoperative, individualised surgical planning with complete free margins is pivotal along with a multidisciplinary approach and collaboration across highly specialised surgical and medical specialties. The present review gives a structured overview of the most common forms of dermal sarcomas including surgical recommendations and examples for advanced reconstructions as well as the current adjunctive medical treatment strategies. Optimal aesthetic and functional outcomes with low recurrence rates can be achieved by using a multidisciplinary approach to complex dermal sarcomas. In cases of extended local tumour invasion in dermal sarcomas, advanced reconstructive techniques can be applied, and the interdisciplinary microsurgeon should be an integral part of the sarcoma board.<\/jats:p>","DOI":"10.3390\/cancers14071693","type":"journal-article","created":{"date-parts":[[2022,3,27]],"date-time":"2022-03-27T21:27:51Z","timestamp":1648416471000},"page":"1693","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A Multidisciplinary Approach to Complex Dermal Sarcomas Ensures an Optimal Clinical Outcome"],"prefix":"10.3390","volume":"14","author":[{"given":"Hannah","family":"Tr\u00f8strup","sequence":"first","affiliation":[{"name":"Department of Plastic Surgery and Breast Surgery, Zealand University Hospital (SUH) Roskilde, University of Copenhagen, 4000 Roskilde, Denmark"},{"name":"Department of Plastic Surgery and Burns Treatment, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8852-5132","authenticated-orcid":false,"given":"Amir K.","family":"Bigdeli","sequence":"additional","affiliation":[{"name":"Department of Hand, Plastic, and Reconstructive Surgery, BG Trauma Center Ludwigshafen, Heidelberg University, D-67071 Ludwigshafen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Krogerus","sequence":"additional","affiliation":[{"name":"Department of Plastic Surgery and Breast Surgery, Zealand University Hospital (SUH) Roskilde, University of Copenhagen, 4000 Roskilde, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Kneser","sequence":"additional","affiliation":[{"name":"Department of Hand, Plastic, and Reconstructive Surgery, BG Trauma Center Ludwigshafen, Heidelberg University, D-67071 Ludwigshafen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grethe","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Plastic Surgery and Burns Treatment, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volker J.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Plastic Surgery and Breast Surgery, Zealand University Hospital (SUH) Roskilde, University of Copenhagen, 4000 Roskilde, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1002\/ijc.2910330108","article-title":"Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system","volume":"33","author":"Trojani","year":"1984","journal-title":"Int. J. Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1002\/cncr.23571","article-title":"Cutaneous soft tissue sarcoma incidence patterns in the U.S.: An analysis of 12,114 cases","volume":"113","author":"Rouhani","year":"2008","journal-title":"Cancer"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1097\/00000478-200604000-00002","article-title":"The Prognostic Significance of Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans","volume":"30","author":"Abbott","year":"2006","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1001\/jamadermatol.2016.1886","article-title":"Prognostic Factors, Treatment, and Survival in Dermatofibrosarcoma Protuberans","volume":"152","author":"Criscito","year":"2016","journal-title":"JAMA Dermatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"8478","DOI":"10.18632\/oncotarget.23899","article-title":"EGFR is involved in dermatofibrosarcoma protuberans progression to high grade sarcoma","volume":"9","author":"Osio","year":"2018","journal-title":"Oncotarget"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1001\/jamadermatol.2015.2389","article-title":"A Novel Chromosomal Translocation Associated with COL1A2-PDGFBGene Fusion in Dermatofibrosarcoma Protuberans: PDGF Expression as a New Diagnostic Tool","volume":"151","author":"Nakamura","year":"2015","journal-title":"JAMA Dermatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/j.det.2019.05.006","article-title":"Dermatofibrosarcoma Protuberans","volume":"37","author":"Allen","year":"2019","journal-title":"Dermatol. Clin."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1064","DOI":"10.1016\/j.jaad.2016.06.018","article-title":"Epidemiology of Bednar tumors in the United States","volume":"75","author":"Liszewski","year":"2016","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/0190-9622(95)90132-9","article-title":"Surgical margins for excision of dermatofibrosarcoma protuberans","volume":"32","author":"Parker","year":"1995","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1772","DOI":"10.1200\/JCO.2009.25.7899","article-title":"Imatinib Mesylate in Advanced Dermatofibrosarcoma Protuberans: Pooled Analysis of Two Phase II Clinical Trials","volume":"28","author":"Rutkowski","year":"2010","journal-title":"J. Clin. Oncol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2604","DOI":"10.1016\/j.ejca.2015.06.108","article-title":"Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline","volume":"51","author":"Saiag","year":"2015","journal-title":"Eur. J. Cancer"},{"key":"ref_12","first-page":"663","article-title":"S1 guidelines for dermatofibrosarcoma protuberans (DFSP)\u2014Update 2018","volume":"17","author":"Ugurel","year":"2019","journal-title":"JDDG J. Dtsch. Dermatol. Ges."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1002\/micr.30640","article-title":"Microsurgical reconstruction of extensive lower extremity defects with the conjoined parascapular and latissimus dorsi free flap","volume":"40","author":"Bigdeli","year":"2020","journal-title":"Microsurgery"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1097\/PAS.0000000000000210","article-title":"Sarcoma-like Tumor of Head and Neck Skin","volume":"38","author":"Nonaka","year":"2014","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1097\/DAD.0b013e3181c80b97","article-title":"Atypical Fibroxanthoma: A Histological and Immunohistochemical Review of 171 Cases","volume":"32","author":"Beer","year":"2010","journal-title":"Am. J. Dermatopathol."},{"key":"ref_16","first-page":"19","article-title":"CGH analysis shows genetic similarities and differences in atypical fibroxanthoma and undifferentiated high. grade pleomorphic sarcoma","volume":"24","author":"Zhao","year":"2004","journal-title":"Anticancer Res."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1053\/hupa.2001.28953","article-title":"H-, K-, and N-ras gene mutation in atypical fibroxanthoma and malignant fibrous histiocytoma","volume":"32","author":"Sakamoto","year":"2001","journal-title":"Hum. Pathol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1136\/jcp.56.9.666","article-title":"Expression of smooth muscle markers in so called malignant fibrous histiocytomas","volume":"56","author":"Hasegawa","year":"2003","journal-title":"J. Clin. Pathol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1097\/PAS.0b013e31825359e1","article-title":"Pleomorphic Dermal Sarcoma: Adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma","volume":"36","author":"Miller","year":"2012","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1111\/cup.13346","article-title":"Immunohistochemical expression of melanocytic and myofibroblastic markers and their molecular correlation in atypical fibroxanthomas and pleomorphic dermal sarcomas","volume":"45","author":"Helbig","year":"2018","journal-title":"J. Cutan. Pathol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1038\/modpathol.2013.168","article-title":"TERT promoter mutations are frequent in atypical fibroxanthomas and pleomorphic dermal sarcomas","volume":"27","author":"Griewank","year":"2014","journal-title":"Mod. Pathol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1038\/modpathol.2017.146","article-title":"Atypical fibroxanthoma and pleomorphic dermal sarcoma harbor frequent NOTCH1\/2 and FAT1 mutations and similar DNA copy number alteration profiles","volume":"31","author":"Griewank","year":"2018","journal-title":"Mod. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"21763","DOI":"10.18632\/oncotarget.7845","article-title":"Oncogene and therapeutic target analyses in atypical fibroxanthomas and pleomorphic dermal sarcomas","volume":"7","author":"Helbig","year":"2016","journal-title":"Oncotarget"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1016\/j.jaad.2018.05.022","article-title":"Undifferentiated pleomorphic sarcoma: Factors predictive of adverse outcomes","volume":"79","author":"Winchester","year":"2018","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1111\/jdv.15493","article-title":"Extended surgical safety margins and ulceration are associated with an improved prognosis in pleomorphic dermal sarcomas","volume":"33","author":"Persa","year":"2019","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1002\/1097-0142(19810101)47:1<156::AID-CNCR2820470127>3.0.CO;2-#","article-title":"Leiomyosarcoma of the skin and subcutaneous tissue","volume":"47","author":"Fields","year":"1981","journal-title":"Cancer"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1097\/00000478-199709000-00001","article-title":"Cutaneous Leiomyosarcoma","volume":"21","author":"Kaddu","year":"1997","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1111\/j.1365-2559.2009.03471.x","article-title":"Primary cutaneous leiomyosarcoma: Clinicopathological analysis of 36 cases","volume":"56","author":"Massi","year":"2010","journal-title":"Histopathology"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1038\/s41572-019-0060-9","article-title":"Kaposi sarcoma","volume":"5","author":"Cesarman","year":"2019","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1002\/path.4441","article-title":"The role of Kaposi sarcoma-associated herpesvirus in the pathogenesis of Kaposi sarcoma","volume":"235","author":"Gramolelli","year":"2014","journal-title":"J. Pathol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1097\/00000372-197900120-00011","article-title":"Subtle clues to diagnosis by conventional microscopy. The patch stage of Kaposi\u2019s sarcoma","volume":"1","author":"Ackerman","year":"1979","journal-title":"Am. J. Dermatopathol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1038\/modpathol.3800061","article-title":"Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in the diagnosis of Kaposi sarcoma","volume":"17","author":"Patel","year":"2004","journal-title":"Mod. Pathol."},{"key":"ref_33","first-page":"2","article-title":"Brief S1 guidelines\u2014Cutaneous angiosarcoma and Kaposi sarcoma","volume":"11","author":"Vogt","year":"2013","journal-title":"JDDG J. Dtsch. Dermatol. Ges."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/S0936-6555(05)80269-9","article-title":"Liposomal doxorubicin (Doxil): An effective new treatment for Kaposi\u2019s sarcoma in AIDS","volume":"6","author":"James","year":"1994","journal-title":"Clin. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.anndiagpath.2010.07.012","article-title":"Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973\u20132007","volume":"15","author":"Schwartz","year":"2011","journal-title":"Ann. Diagn. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1002\/jso.20766","article-title":"Detection and characterization of vascular endothelial growth factors and their receptors in a series of angiosarcomas","volume":"97","author":"Itakura","year":"2008","journal-title":"J. Surg. Oncol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1097\/PAP.0b013e31826661d1","article-title":"Cutaneous Soft Tissue Tumors That Make You Say, \u201cOh $*&%!.\u201d","volume":"19","author":"Patel","year":"2012","journal-title":"Adv. Anat. Pathol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1136\/jclinpath-2017-204601","article-title":"Cutaneous angiosarcoma: A current update","volume":"70","author":"Shustef","year":"2017","journal-title":"J. Clin. Pathol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1016\/j.ad.2018.06.013","article-title":"Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment","volume":"109","author":"Requena","year":"2018","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1097\/CCO.0000000000000427","article-title":"Cutaneous angiosarcoma: Update on biology and latest treatment","volume":"30","author":"Ishida","year":"2018","journal-title":"Curr. Opin. Oncol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1007\/s12105-018-0978-3","article-title":"Characteristics and Prognosis of Primary Head and Neck Angiosarcomas: A Surveillance, Epidemiology, and End Results Program (SEER) Analysis of 1250 Cases","volume":"13","author":"Lee","year":"2018","journal-title":"Head Neck Pathol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1067\/mjd.2002.120909","article-title":"Lymphedema: An immunologically vulnerable site for development of neoplasms","volume":"47","author":"Ruocco","year":"2002","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1097\/01.pas.0000126055.33916.0b","article-title":"Cutaneous Angiosarcoma Following Breast-conserving Surgery and Radiation: An Analysis of 27 Cases","volume":"28","author":"Billings","year":"2004","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1097\/SLA.0000000000001753","article-title":"Cutaneous Radiation-associated Breast Angiosarcoma: Radicality of Surgery Impacts Survival","volume":"265","author":"Li","year":"2017","journal-title":"Ann. Surg."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1111\/cup.13912","article-title":"MYC gene amplification by fluorescence in situ hybridization and MYC protein expression by immunohistochemistry in the diagnosis of cutaneous angiosarcoma: Systematic review and appropriate use criteria","volume":"48","author":"Motaparthi","year":"2021","journal-title":"J. Cutan. Pathol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1002\/bjs.1800750817","article-title":"Reconstruction after excision of soft tissue sarcomas of the limbs and trunk","volume":"75","author":"Stotter","year":"2005","journal-title":"Br. J. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"L\u00f3pez, J.F., Hietanen, K.E., Kaartinen, I.S., K\u00e4\u00e4ri\u00e4inen, M.T., Pakarinen, T.-K., Laitinen, M., and Kuokkanen, H. (2015). Primary flap reconstruction of tissue defects after sarcoma surgery enables curative treatment with acceptable functional results: A 7-year review. BMC Surg., 15.","DOI":"10.1186\/s12893-015-0060-y"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1245\/ASO.2004.06.026","article-title":"Upper Extremity Reconstruction Following Resection of Soft Tissue Sarcomas: A Functional Outcomes Analysis","volume":"11","author":"Kim","year":"2004","journal-title":"Ann. Surg. Oncol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.jvs.2015.02.004","article-title":"Surgical outcomes of vascular reconstruction in soft tissue sarcomas of the lower extremities","volume":"62","author":"Nishinari","year":"2015","journal-title":"J. Vasc. Surg."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"2256","DOI":"10.1002\/cncr.10419","article-title":"Soft tissue sarcoma of the upper extremity: A 5-year ex-perience at two institutions emphasizing the role of soft tissue flap reconstruction","volume":"94","author":"Lohman","year":"2002","journal-title":"Cancer"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/j.jvs.2016.05.094","article-title":"Limb-sparing surgery with vascular reconstruction for malignant lower extremity soft tissue sarcoma","volume":"65","author":"Davis","year":"2017","journal-title":"J. Vasc. Surg."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.ejso.2003.12.005","article-title":"Outcomes of surgery for dermatofibrosarcoma protuberans","volume":"30","author":"Chang","year":"2004","journal-title":"Eur. J. Surg. Oncol. (EJSO)"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"2112","DOI":"10.1245\/s10434-010-1046-8","article-title":"Dermatofibrosarcoma Protuberans: How Wide Should We Resect?","volume":"17","author":"Farma","year":"2010","journal-title":"Ann. Surg. Oncol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1097\/DSS.0000000000000910","article-title":"A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience","volume":"43","author":"Lowe","year":"2017","journal-title":"Dermatol. Surg."},{"key":"ref_55","first-page":"82","article-title":"A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans","volume":"35","author":"Gloster","year":"1996","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_56","first-page":"1607035","article-title":"Dermatofibrosarcoma protuberans: Treatment and prognosis","volume":"18","author":"Rutgers","year":"1992","journal-title":"Eur. J. Surg. Oncol. (EJSO)"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/j.bjps.2015.10.027","article-title":"Long-term outcomes of surgical treatment for dermatofibrosarcoma protuberans according to width of gross resection margin","volume":"69","author":"Woo","year":"2016","journal-title":"J. Plast. Reconstr. Aesthetic Surg."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1016\/j.bjps.2020.02.012","article-title":"Clinical and pathological analysis of dermatofibrosarcoma protuberans with long-term follow-up","volume":"73","author":"Shi","year":"2020","journal-title":"J. Plast. Reconstr. Aesthetic Surg."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1111\/cup.12603","article-title":"Pleomorphic dermal sarcoma: A more aggressive neoplasm than previously estimated","volume":"43","author":"Pinedo","year":"2016","journal-title":"J. Cutan. Pathol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1111\/cup.13787","article-title":"Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal","volume":"48","author":"Cesinaro","year":"2021","journal-title":"J. Cutan. Pathol."},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Jibbe, A., Worley, B., Miller, C.H., and Alam, M. (2021). Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review. J. Am. Acad. Dermatol.","DOI":"10.1016\/j.jaad.2021.09.036"},{"key":"ref_62","first-page":"2507642","article-title":"Electrochemotherapy as Promising Treatment Option in Rare Recurrent Cutaneous Neoplasm of the Scalp: Case Report of an Elderly Patient","volume":"2019","author":"Crimini","year":"2019","journal-title":"Case Rep. Oncol. Med."},{"key":"ref_63","first-page":"4","article-title":"Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment","volume":"110","author":"Llombart","year":"2018","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/j.jaad.2014.07.020","article-title":"Leiomyosarcoma of the skin: Clinical, histopathologic, and prognostic factors that influence outcomes","volume":"71","author":"Winchester","year":"2014","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1002\/jso.24352","article-title":"A prognostic model for resectable soft tissue and cutaneous angiosarcoma","volume":"114","author":"Sinnamon","year":"2016","journal-title":"J. Surg. Oncol."},{"key":"ref_66","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_67","doi-asserted-by":"crossref","unstructured":"Wiegand, S., Dietz, A., and Wichmann, G. (2021). Malignant Vascular Tumors of the Head and Neck\u2014Which Type of Therapy Works Best?. Cancers, 13.","DOI":"10.3390\/cancers13246201"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1002\/hed.21513","article-title":"Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp","volume":"33","author":"Guadagnolo","year":"2010","journal-title":"Head Neck"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1055\/s-0043-106583","article-title":"Strahlen-induzierte Angiosarkome der Brust [Radiation-induced angiosarcoma of the breast]","volume":"49","author":"Lehnhardt","year":"2017","journal-title":"Handchir. Mikrochir. Plast. Chir."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.3109\/0284186X.2014.983657","article-title":"Radiation-associated angiosarcoma after breast cancer: Improved survival by excision of all irradiated skin and soft tissue of the thoracic wall? A report of six patients","volume":"54","author":"Styring","year":"2014","journal-title":"Acta Oncol."},{"key":"ref_71","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_72","doi-asserted-by":"crossref","first-page":"2235","DOI":"10.1016\/S0140-6736(02)09292-9","article-title":"Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: A randomised trial","volume":"359","author":"Davis","year":"2002","journal-title":"Lancet"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1016\/j.jaad.2018.08.054","article-title":"Prognostic factors, treatment, and survival in cutaneous pleomorphic sarcoma","volume":"83","author":"Ibanez","year":"2020","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1200\/JCO.1993.11.7.1269","article-title":"Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas","volume":"11","author":"Edmonson","year":"1993","journal-title":"J. Clin. Oncol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1023\/A:1012272007146","article-title":"Docetaxel as rescue medication in anthracycline- and ifosfamide-resistant locally advanced or metastatic soft tissue sarcoma: Results of a phase II trial","volume":"12","author":"Brodowicz","year":"2001","journal-title":"Ann. Oncol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"2952","DOI":"10.1002\/cncr.30191","article-title":"Chemotherapy for soft tissue sarcoma","volume":"122","author":"Ratan","year":"2016","journal-title":"Cancer"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1097\/00000658-198209000-00009","article-title":"The Treatment of Soft-tissue Sarcomas of the Extremities: Prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy","volume":"196","author":"Rosenberg","year":"1982","journal-title":"Ann. Surg."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1002\/1097-0142(19830801)52:3<424::AID-CNCR2820520307>3.0.CO;2-8","article-title":"Prospective randomized evaluation of adjuvant chemotherapy in adults with soft tissue sarcomas of the extremities","volume":"52","author":"Rosenberg","year":"1983","journal-title":"Cancer"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1016\/S0360-3016(03)00186-X","article-title":"Neoadjuvant chemotherapy and radiotherapy for large extremity soft-tissue sarcomas","volume":"56","author":"DeLaney","year":"2003","journal-title":"Int. J. Radiat. Oncol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"3758","DOI":"10.1002\/cncr.26696","article-title":"Long-term follow-up of patients treated with neoadjuvant chemotherapy and radiotherapy for large, extremity soft tissue sarcomas","volume":"118","author":"Mullen","year":"2012","journal-title":"Cancer"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"3288","DOI":"10.1158\/1078-0432.CCR-09-3401","article-title":"Imatinib Mesylate as a Preoperative Therapy in Dermatofibrosarcoma: Results of a Multicenter Phase II Study on 25 Patients","volume":"16","author":"Porcher","year":"2010","journal-title":"Clin. Cancer Res."},{"key":"ref_82","doi-asserted-by":"crossref","unstructured":"Beaziz, J., Battistella, M., Delyon, J., Farges, C., Marco, O., Pages, C., Le Maignan, C., da Meda, L., Basset-Seguin, N., and Resche-Rigon, M. (2021). Long-Term Outcome of Neoadjuvant Tyrosine Kinase Inhibitors Followed by Complete Surgery in Locally Advanced Dermatofibrosarcoma Protuberans. Cancers, 13.","DOI":"10.3390\/cancers13092224"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"e001580","DOI":"10.1136\/jitc-2020-001580","article-title":"Immunotherapy for sarcomas: New frontiers and unveiled opportunities","volume":"9","author":"Birdi","year":"2021","journal-title":"J. Immunother. Cancer"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1016\/S1470-2045(17)30624-1","article-title":"Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): A multicentre, two-cohort, single-arm, open-label, phase 2 trial","volume":"18","author":"Tawbi","year":"2017","journal-title":"Lancet Oncol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1038\/s41586-019-1906-8","article-title":"B cells are associated with survival and immunotherapy response in sarcoma","volume":"577","author":"Petitprez","year":"2020","journal-title":"Nature"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1186\/s40425-019-0689-7","article-title":"Angiosarcoma patients treated with immune checkpoint inhibitors: A case series of seven patients from a single institution","volume":"7","author":"Florou","year":"2019","journal-title":"J. Immunother. 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