{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T20:30:59Z","timestamp":1785184259511,"version":"3.55.0"},"reference-count":18,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T00:00:00Z","timestamp":1753056000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>BAP1\u2010inactivated melanocytic tumor is a distinct entity with loss of BAP1 protein and epithelioid morphology. It shares histopathologic features with Spitz nevus and nevoid melanoma, and it can occur sporadically or with germline BAP1 predisposition syndrome. These lesions typically have tumor\u2010infiltrating lymphocytes and infrequent mitoses. They are generally indolent, though melanoma can arise in both germline and sporadic cases. Most show <jats:italic>BRAF<\/jats:italic> V600E and <jats:italic>BAP1<\/jats:italic> mutations. We describe four tumors in one patient diagnosed with BAP1\u2010tumor predisposition syndrome (BAP1\u2010TPDS): two invasive melanomas arising in BIMT and two BIMTs with uncertain malignant potential. Molecular analysis and fluorescence in\u00a0situ hybridization (FISH) revealed <jats:italic>BAP1<\/jats:italic> and <jats:italic>NRAS<\/jats:italic> mutations in melanoma and BAP1\u2010inactivated melanocytic tumor components, with a gain of 6p25 (<jats:italic>RREB1<\/jats:italic>) in the melanoma component only. The patient completed pembrolizumab adjuvant therapy with no evidence of metastasis. This is a rare presentation of BIMT with <jats:italic>BAP1<\/jats:italic> and <jats:italic>NRAS<\/jats:italic> mutations, absence of <jats:italic>BRAF<\/jats:italic> V600 mutation, and loss of BAP1 immunoreactivity in all lesional cells. Our case adds to the understanding of the histomorphologic and mutational spectrum in BAP1\u2010inactivated melanocytic tumors.<\/jats:p>","DOI":"10.1111\/cup.14839","type":"journal-article","created":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T09:54:27Z","timestamp":1753091667000},"page":"627-635","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Invasive Melanoma Arising in a <scp>BAP1<\/scp>\u2010Inactivated Melanocytic Tumor With <i>NRAS<\/i> Mutation: A Report of Exceptional Case With Emphasis on Its Genomic Features and Review of the Literature"],"prefix":"10.1111","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-6656-5587","authenticated-orcid":false,"given":"Muath","family":"Alyahya","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nyi Nyi","family":"May\u2010Phyo","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Kingston Health Sciences Centre and Queen's University  Kingston Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4143-2657","authenticated-orcid":false,"given":"Ami","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Kingston Health Sciences Centre and Queen's University  Kingston Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shamini","family":"Selvarajah","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cuihong","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Calvin","family":"Tseng","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tao","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Kingston Health Sciences Centre and Queen's University  Kingston Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tara","family":"Baetz","sequence":"additional","affiliation":[{"name":"Department of Oncology Kingston Health Sciences Centre and Queen's University  Kingston Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1067-9881","authenticated-orcid":false,"given":"Zaid Saeed","family":"Kamil","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,7,21]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.910"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2018.05.005"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1194472"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2017.2330"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182498be5"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000209"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-014-1674-7"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379\u2010021\u201000976\u20107"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000187"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318263648c"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cll.2017.05.003"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.15434"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2015.30"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.41.2965"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/DAD.0000000000001217"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1383125"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.14346"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2017.12.022"}],"container-title":["Journal of Cutaneous Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cup.14839","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,2]],"date-time":"2025-09-02T00:21:38Z","timestamp":1756772498000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/cup.14839"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,21]]},"references-count":18,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2025,10]]}},"alternative-id":["10.1111\/cup.14839"],"URL":"https:\/\/doi.org\/10.1111\/cup.14839","archive":["Portico"],"relation":{},"ISSN":["0303-6987","1600-0560"],"issn-type":[{"value":"0303-6987","type":"print"},{"value":"1600-0560","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,21]]},"assertion":[{"value":"2025-01-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}