{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:19:04Z","timestamp":1791231544428,"version":"4.1.0"},"reference-count":41,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2023,7,12]],"date-time":"2023-07-12T00:00:00Z","timestamp":1689120000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Sarcoma may show similarities to malignant melanoma in terms of morphologic and immunohistochemical aspects, making it difficult to differentiate between these two neoplasms during the diagnostic process. This systematic review aims to summarize available evidence on cases of sarcoma that were initially diagnosed as melanoma. Methods: A comprehensive search of the MEDLINE\/Pubmed, EMBASE, and SCOPUS databases was conducted through March 2023. We included case series and case reports of sarcoma patients that were initially diagnosed as malignant melanoma. PRISMA guidelines were followed. Results: Twenty-three case reports and four case series with a total of 34 patients were included. The clinical presentation was heterogeneous, and the most involved anatomical regions were lower limbs (24%), head\/neck (24%), and upper limbs (21%). IHC positivity was reported for S100 (69%), HMB45 (63%), MelanA (31%), and MiTF (3%). The main reasons for a second assessment were unusual presentation (48%) and uncertain diagnosis (28%). EWSR1 translocation was investigated in 17\/34 patients (50%) and found to be positive in 16\/17 (94%). The final diagnosis was clear cell sarcoma (50%) or other soft tissue sarcomas (50%). Conclusions: Melanoma and some histotypes of sarcoma share many similarities. In cases of atypical lesions, a second diagnosis should be considered, and ESWR1 translocation should be investigated.<\/jats:p>","DOI":"10.3390\/cancers15143584","type":"journal-article","created":{"date-parts":[[2023,7,13]],"date-time":"2023-07-13T01:58:11Z","timestamp":1689213491000},"page":"3584","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Soft Tissue Sarcoma Mimicking Melanoma: A Systematic Review"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5014-1122","authenticated-orcid":false,"given":"Fortunato","family":"Cassalia","sequence":"first","affiliation":[{"name":"Unit of Dermatology, Department of Medicine, University of Padua, 35121 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0831-3971","authenticated-orcid":false,"given":"Francesco","family":"Cavallin","sequence":"additional","affiliation":[{"name":"Independent Researcher, 36020 Solagna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Danese","sequence":"additional","affiliation":[{"name":"Unit of Dermatology, Department of Integrated Medical and General Activity, University of Verona, 37100 Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2862-8014","authenticated-orcid":false,"given":"Paolo","family":"Del Fiore","sequence":"additional","affiliation":[{"name":"Soft-Tissue, Peritoneum and Melanoma Surgical Oncology Unit, Veneto Institute of Oncology IOV-IRCCS, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Di Prata","sequence":"additional","affiliation":[{"name":"Department of Surgical, Oncological and Gastroenterological Sciences (DISCOG), University of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Rastrelli","sequence":"additional","affiliation":[{"name":"Soft-Tissue, Peritoneum and Melanoma Surgical Oncology Unit, Veneto Institute of Oncology IOV-IRCCS, 35128 Padua, Italy"},{"name":"Department of Surgical, Oncological and Gastroenterological Sciences (DISCOG), University of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5791-0775","authenticated-orcid":false,"given":"Anna","family":"Belloni Fortina","sequence":"additional","affiliation":[{"name":"Pediatric Dermatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Mocellin","sequence":"additional","affiliation":[{"name":"Soft-Tissue, Peritoneum and Melanoma Surgical Oncology Unit, Veneto Institute of Oncology IOV-IRCCS, 35128 Padua, Italy"},{"name":"Department of Surgical, Oncological and Gastroenterological Sciences (DISCOG), University of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2493","DOI":"10.1016\/j.ejca.2011.08.008","article-title":"Rare cancers are not so rare: The rare cancer burden in Europe","volume":"47","author":"Gatta","year":"2011","journal-title":"Eur. J. Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"70","DOI":"10.32074\/1591-951X-213","article-title":"The 2020 WHO Classification of Soft Tissue Tumours: News and perspectives","volume":"113","author":"Sbaraglia","year":"2021","journal-title":"Pathologica"},{"key":"ref_3","unstructured":"(2023, April 10). American Cancer Society Key Statistics for Soft Tissue Sarcomas. Available online: https:\/\/www.cancer.org\/cancer\/soft-tissue-sarcoma\/about\/key-statistics.html."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1097\/PAS.0b013e31814b18fb","article-title":"Clear cell sarcoma of soft tissue: A clinicopathologic, immunohistochemical, and molecular analysis of 33 cases","volume":"32","author":"Hisaoka","year":"2008","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.cancergencyto.2004.04.008","article-title":"Absence of mutations of the BRAF gene in malignant melanoma of soft parts (clear cell sarcoma of tendons and aponeuroses)","volume":"156","author":"Panagopoulos","year":"2005","journal-title":"Cancer Genet. Cytogenet."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1038\/modpathol.3800503","article-title":"Dual-color, break-apart fluorescence in situ hybridization for EWS gene rearrangement distinguishes clear cell sarcoma of soft tissue from malignant melanoma","volume":"18","author":"Patel","year":"2005","journal-title":"Mod. Pathol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"152","DOI":"10.5858\/2007-131-152-CCSOTA","article-title":"Clear cell sarcoma of tendons and aponeuroses: A review","volume":"131","author":"Dim","year":"2007","journal-title":"Arch. Pathol. Lab Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1412","DOI":"10.1002\/1097-0142(19841001)54:7<1412::AID-CNCR2820540730>3.0.CO;2-A","article-title":"Clear-cell sarcoma of tendons and aponeuroses: A clinicopathologic study. Presentation of six additional cases with review of the literature","volume":"54","author":"Pavlidis","year":"1984","journal-title":"Cancer"},{"key":"ref_9","first-page":"e13424","article-title":"A Rare Case of Epithelioid Malignant Peripheral Nerve Sheath Tumor Mimicking Malignant Melanoma","volume":"13","author":"Biglow","year":"2021","journal-title":"Cureus"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1111\/his.13740","article-title":"Evaluation of eight melanocytic and neural crest-associated markers in well-characterized series of 124 malignant peripheral nerve sheath tumours (MPNST): Useful to distinguish MPNST from melanoma?","volume":"73","author":"Gaspard","year":"2018","journal-title":"Histopathology"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"4514","DOI":"10.3748\/wjg.v13.i33.4514","article-title":"Intestinal Kaposi\u2019s sarcoma may mimic gastrointestinal stromal tumor in HIV infection","volume":"13","author":"Zoufaly","year":"2007","journal-title":"World J. Gastroenterol."},{"key":"ref_12","first-page":"e26086","article-title":"The Outcome of Extremity Soft Tissue Sarcomas in Terms of Resection Margins: A Study from a Cancer Dedicated Center","volume":"14","author":"Shafiq","year":"2022","journal-title":"Cureus"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"101007","DOI":"10.1016\/j.adro.2022.101007","article-title":"Radiation Therapy Before Repeat Wide Resection for Unplanned Surgery of Soft Tissue Sarcoma (\u201cOops\u201d Operation) Results in Improved Disease-Free Survival","volume":"7","author":"Kollender","year":"2022","journal-title":"Adv. Radiat. Oncol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1245\/s10434-022-12688-6","article-title":"Sentinel Lymph Node Biopsy for Extremity and Truncal Soft Tissue Sarcomas: A Systematic Review of the Literature","volume":"30","author":"Keung","year":"2023","journal-title":"Ann. Surg. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: An updated guideline for reporting systematic reviews","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"ref_16","first-page":"2127","article-title":"Methodological quality of case series studies: An introduction to the JBI critical appraisal tool","volume":"18","author":"Munn","year":"2020","journal-title":"JBI Evid. Synth."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1016\/j.pathol.2021.05.097","article-title":"Cutaneous clear cell sarcoma with an epidermal component mimicking melanoma","volume":"54","author":"Potter","year":"2022","journal-title":"Pathology"},{"key":"ref_18","first-page":"e32010","article-title":"Clear-Cell Sarcoma with an Unusual Presentation Mimicking Metastatic Melanoma","volume":"14","author":"Elousrouti","year":"2022","journal-title":"Cureus"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"200489","DOI":"10.1016\/j.ehpc.2021.200489","article-title":"Atypical clear cell sarcoma of the pleura presenting as large pleural effusion with 22q12 abnormality: A challenging case with twists and turns","volume":"24","author":"Zhang","year":"2021","journal-title":"Hum. Pathol. Case Rep."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1111\/cup.13659","article-title":"A rare case of primary dermal clear cell sarcoma with focal epidermotropism: An entity difficult to distinguish from melanoma","volume":"47","author":"Nawrocki","year":"2020","journal-title":"J. Cutan. Pathol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"213","DOI":"10.5306\/wjco.v10.i5.213","article-title":"Clear cell sarcoma in unusual sites mimicking metastatic melanoma","volume":"10","author":"Obiorah","year":"2019","journal-title":"World J. Clin. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.pathol.2018.10.019","article-title":"Clear cell sarcoma of the soft palate mimicking unclassified melanoma","volume":"51","author":"Donzel","year":"2019","journal-title":"Pathology"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"203","DOI":"10.4274\/balkanmedj.2017.0796","article-title":"Primary Clear Cell Sarcoma of the Dermis Mimicking Malignant Melanoma","volume":"35","author":"Obiorah","year":"2018","journal-title":"Balkan Med. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1097\/DAD.0000000000001150","article-title":"Case Report of Myeloid Sarcoma Masquerading as In-Transit Metastasis at a Previous Melanoma Site: Avoiding a Diagnostic Pitfall","volume":"40","author":"Curry","year":"2018","journal-title":"Am. J. Dermatopathol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1111\/cup.12968","article-title":"Index report of cutaneous angiosarcomas with strong positivity for tyrosinase mimicking melanoma with further evaluation of melanocytic markers in a large angiosarcoma series","volume":"44","author":"Chrisinger","year":"2017","journal-title":"J. Cutan. Pathol."},{"key":"ref_26","first-page":"S1","article-title":"Cutaneous clear cell sarcoma with intraepidermal component mimicking spitzoid melanoma","volume":"471","author":"Zivanovic","year":"2017","journal-title":"Virchows Arch."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1111\/cup.12834","article-title":"Superficial malignant peripheral nerve sheath tumor with overlying intradermal melanocytic nevus mimicking spindle cell melanoma","volume":"43","author":"Jackson","year":"2016","journal-title":"J. Cutan. Pathol."},{"key":"ref_28","first-page":"S2239253X15023701","article-title":"Unusual bleeding of a giant cell fibroblastoma: A soft tissue sarcoma of the skin mimicking metastatic melanoma","volume":"86","author":"Castriconi","year":"2015","journal-title":"Ann. Ital. Chir."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"A409","DOI":"10.1093\/ajcp\/144.suppl2.409","article-title":"Colonic Clear Cell Sarcoma Associated with Neurofibromatosis Type 2","volume":"144","author":"Sayah","year":"2015","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_30","first-page":"5242","article-title":"Absence of 19 known hotspot oncogenic mutations in soft tissue clear cell sarcoma: Two cases report with review of the literature","volume":"7","author":"Liu","year":"2014","journal-title":"Int. J. Clin. Exp. Pathol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1111\/cup.12058","article-title":"Superficial paramucosal clear cell sarcoma of the soft parts resembling melanoma in a 13-year-old boy","volume":"40","author":"Sidiropoulos","year":"2013","journal-title":"J. Cutan. Pathol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1097\/DAD.0b013e3182473190","article-title":"Cutaneous clear cell sarcoma: Report of three cases of a potentially underestimated mimicker of spindle cell melanoma","volume":"34","author":"Falconieri","year":"2012","journal-title":"Am. J. Dermatopathol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"168","DOI":"10.4103\/0019-5154.53193","article-title":"Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma","volume":"54","author":"Esquivel","year":"2009","journal-title":"Indian J. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"e26","DOI":"10.4081\/rt.2009.e26","article-title":"Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation","volume":"1","author":"Tanas","year":"2009","journal-title":"Rare Tumors"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1111\/j.0303-6987.2006.00387.x","article-title":"Macrophage-rich epithelioid angiosarcoma mimicking malignant melanoma","volume":"33","author":"Brightman","year":"2006","journal-title":"J. Cutan. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.prp.2005.01.008","article-title":"Epithelioid malignant peripheral nerve sheath tumor. Report of a case with inflammatory infiltration","volume":"201","author":"Matsuda","year":"2005","journal-title":"Pathol. Res. Pract."},{"key":"ref_37","first-page":"879","article-title":"Superficial malignant schwannoma of the scalp","volume":"29","author":"Demir","year":"2003","journal-title":"Dermatol. Surg."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1038\/modpathol.3880351","article-title":"Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis","volume":"14","author":"Bonetti","year":"2001","journal-title":"Mod. Pathol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1097\/00000478-199508000-00006","article-title":"Hyaline-cell rich chondroid syringoma. A tumor mimicking malignancy","volume":"19","author":"Ferreiro","year":"1995","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_40","first-page":"195","article-title":"Epithelioid malignant schwannoma. A case report","volume":"39","author":"Honma","year":"1989","journal-title":"Acta Pathol. Jpn."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1097\/00000372-198906000-00001","article-title":"Malignant giant cell tumor of soft parts presenting as a skin tumor","volume":"11","author":"Gould","year":"1989","journal-title":"Am. J. Dermatopathol."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/15\/14\/3584\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:11:24Z","timestamp":1760127084000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/15\/14\/3584"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,12]]},"references-count":41,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2023,7]]}},"alternative-id":["cancers15143584"],"URL":"https:\/\/doi.org\/10.3390\/cancers15143584","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,7,12]]}}}