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Herein we present a case of a 60\u2010year\u2010old female with a 3.8\u00a0cm presacral mass that was diagnosed as a MMNST. A molecular workup demonstrated <jats:italic>TERT<\/jats:italic> promoter \u2010124C\u2009&gt;\u2009T and <jats:italic>TET2 Q891<\/jats:italic>* gene mutations. Within 2\u2009years of her initial diagnosis, she had developed widespread metastasis and pleural effusions. A cytologic workup of the pleural fluid revealed clusters of vacuolated epithelioid cells with enlarged nuclei, prominent nucleoli, and occasional multinucleation. The lesional cells were positive for SOX10, S100\u2010protein, Melan\u2010A, and HMB45, while negative for Calretinin, MOC31, and monoclonal CEA. In this clinicopathologic context, a diagnosis of metastatic MMNST was rendered. Awareness of this entity and its clinical presentation, along with a critical understanding of its molecular findings and that of imitators, is crucial in achieving an accurate diagnosis.<\/jats:p>","DOI":"10.1002\/dc.24895","type":"journal-article","created":{"date-parts":[[2021,10,26]],"date-time":"2021-10-26T09:21:10Z","timestamp":1635240070000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Malignant melanotic nerve sheath tumor in pleural effusion: Deceitful cytology with significant repercussions"],"prefix":"10.1002","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0597-4811","authenticated-orcid":false,"given":"Christopher","family":"Jackson","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Dartmouth\u2010Hitchcock Medical Center  Lebanon New Hampshire USA"},{"name":"Geisel School of Medicine at Dartmouth  Hanover New Hampshire USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konstantinos","family":"Linos","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Dartmouth\u2010Hitchcock Medical Center  Lebanon New Hampshire USA"},{"name":"Geisel School of Medicine at Dartmouth  Hanover New Hampshire USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0597-685X","authenticated-orcid":false,"given":"Xiaoying","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Dartmouth\u2010Hitchcock Medical Center  Lebanon New Hampshire USA"},{"name":"Geisel School of Medicine at Dartmouth  Hanover New Hampshire USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,10,26]]},"reference":[{"key":"e_1_2_9_2_1","volume-title":"Soft Tissue and Bone Tumours","author":"WHO Classification of Tumours Editorial Board","year":"2020"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.1999.00786.x"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.40.12.1449"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.1992.tb00920.x"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(94)31249-8"},{"key":"e_1_2_9_7_1","doi-asserted-by":"crossref","unstructured":"RanjanA ChackoG ChandiSM.Intracerebellar melanotic schwannoma: a case report.2009;9(5):687\u2010690. doi:10.1080\/02688699550041007","DOI":"10.1080\/02688699550041007"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182a0a150"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199003000-00002"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21178"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2008-2333"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.20.3037"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0405535101"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2002.tb04323.x"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22254"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0339(200007)23:1<55::AID-DC13>3.0.CO;2-B"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0339(199804)18:4<284::AID-DC7>3.0.CO;2-C"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0339(199810)19:4<298::AID-DC15>3.0.CO;2-I"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1159\/000325096"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/dc.2840110319"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000328605"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/DAD.0000000000001482"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-12-0095"},{"issue":"905","key":"e_1_2_9_24_1","first-page":"1","article-title":"Is immunohistochemistry of BRAF V600E useful as a screening tool and during progression disease of melanoma patients?","volume":"16","author":"Schirosi L","year":"2016","journal-title":"BMC Cancer"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajco.12574"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001458"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1502583"},{"key":"e_1_2_9_28_1","unstructured":"TERT Gene\u2014Somatic Mutations in Cancer.https:\/\/cancer.sanger.ac.uk\/cosmic\/gene\/analysis?ln=TERT. 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