{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T12:02:38Z","timestamp":1790769758000,"version":"4.1.0"},"reference-count":35,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Several activating mutations and gene fusions involving the mitogen\u2010activated protein kinase (MAPK) pathway have appeared in the literature regarding histiocytic neoplasms. We identified a\n                    <jats:italic>GAB2::BRAF<\/jats:italic>\n                    fusion in a cutaneous lesion of a 36\u2010year\u2010old male who developed central diabetes insipidus and reddish\u2010pink grouped papules on the bilateral axillary rims, inguinal region, and periocular skin. Skin biopsy obtained from an axillary lesion showed a CD68+, S100\u2010, and CD1a\u2010 histiocytic proliferation with foamy histiocytes and multinucleated Touton giant cells, compatible with xanthogranuloma. Next\u2010generation sequencing identified a\n                    <jats:italic>GAB2::BRAF<\/jats:italic>\n                    fusion involving Exon 2 of\n                    <jats:italic>GAB2<\/jats:italic>\n                    and Exon 10 of\n                    <jats:italic>BRAF<\/jats:italic>\n                    . Our case further highlights this novel fusion in the MAPK signaling pathway as a possible driver of nonLangerhans cell histiocytosis (NLCH) and underscores the utility of performing molecular studies on skin biopsy specimens with NLCH to help identify potential targets for therapy.\n                  <\/jats:p>","DOI":"10.1111\/cup.70229","type":"journal-article","created":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:35:48Z","timestamp":1790768148000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Disseminated Xanthogranuloma Harboring a\n                    <i>GAB2::BRAF<\/i>\n                    Fusion and\n                    <i>MITF<\/i>\n                    Pathogenic Variant Successfully Treated With Cobimetinib: A Rare Case Report"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3061-0390","authenticated-orcid":false,"given":"Pauline C.","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Pathology, Microbiology, and Immunology, College of Medicine University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0028-9371","authenticated-orcid":false,"given":"Allison M.","family":"Cushman\u2010Vokoun","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology, and Immunology, College of Medicine University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher R.","family":"D\u2019Angelo","sequence":"additional","affiliation":[{"name":"Division of Medical Oncology and Hematology, Department of Internal Medicine, College of Medicine University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8591-1601","authenticated-orcid":false,"given":"Dinesh","family":"Pradhan","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology, and Immunology, College of Medicine University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,30]]},"reference":[{"issue":"5","key":"e_1_2_8_2_1","first-page":"243","article-title":"Cutaneous Disseminated Xanthogranuloma in an Adult: Case Report and Review of the Literature","volume":"83","author":"Asarch A.","year":"2009","journal-title":"Cutis"},{"key":"e_1_2_8_3_1","first-page":"222","article-title":"Congenital Xanthoma Multipex in a Child","volume":"17","author":"Adamson H.","year":"1905","journal-title":"British Journal of Dermatology"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1912.tb16720.x"},{"key":"e_1_2_8_5_1","first-page":"625","article-title":"Juvenile Xanthogranuloma (Nevoxanthoendothelioma)","volume":"30","author":"Helwig E.","year":"1954","journal-title":"American Journal of Pathology"},{"key":"e_1_2_8_6_1","first-page":"409","article-title":"Nevoxanthoendothelioma With Small and Large Nodules. Report on 13 Cases","volume":"215","author":"Gartmann H.","year":"1963","journal-title":"Archiv f\u00fcr Klinische und Experimentelle Dermatologie"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200305000-00003"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2015.1.571"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2017.50"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-019-0653-6"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(17)30031-1"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24772"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-021-00307-9"},{"issue":"13","key":"e_1_2_8_14_1","doi-asserted-by":"crossref","first-page":"2991","DOI":"10.1182\/bloodadvances.2020001890","article-title":"Dasatinib Induces a Dramatic Response in a Child With Refractory Juvenile Xanthogranuloma With a Novel MRC1\u2010PDGFRB Fusion","volume":"4","author":"Eissa S. S.","year":"2020","journal-title":"Blood Advances"},{"issue":"1","key":"e_1_2_8_15_1","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1186\/s40478-019-0811-6","article-title":"BRAF V600E Mutation in Juvenile Xanthogranuloma Family Neoplasms of the Central Nervous System (CNS\u2010JXG): A Revised Diagnostic Algorithm to Include Pediatric Erdheim\u2010Chester Disease","volume":"7","author":"Picarsic J.","year":"2019","journal-title":"Acta Neuropathologica Communications"},{"issue":"1","key":"e_1_2_8_16_1","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1093\/bjd\/ljae328","article-title":"Disseminated Juvenile Xanthogranuloma Harbouring a GAB2::BRAF Fusion Successfully Treated With Trametinib: A Case Report","volume":"192","author":"Shen K.\u2010n.","year":"2025","journal-title":"British Journal of Dermatology"},{"issue":"8","key":"e_1_2_8_17_1","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1111\/cup.14231","article-title":"A Novel GAB2::BRAF Fusion in Cutaneous Non\u2010Langerhans\u2010Cell Histiocytosis With Systemic Involvement","volume":"49","author":"Wu B.","year":"2022","journal-title":"Journal of Cutaneous Pathology"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1158\/1541-7786.MCR-12-0352"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-019-1012-y"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-020-0944-4"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-13-1746"},{"issue":"6","key":"e_1_2_8_22_1","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1038\/s41408-022-00693-7","article-title":"Clinical and Therapeutic Implications of BRAF Fusions in Histiocytic Disorders","volume":"12","author":"Zanwar S.","year":"2022","journal-title":"Blood Cancer Journal"},{"issue":"1","key":"e_1_2_8_23_1","doi-asserted-by":"crossref","first-page":"6429","DOI":"10.1182\/blood-2023-186973","article-title":"Disseminated Xanthogranuloma With BRAF Gene Fusion and Profound Response to MEK Inhibitor Therapy","volume":"142","author":"Lytvynova O.","year":"2023","journal-title":"Blood"},{"issue":"4","key":"e_1_2_8_24_1","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.3324\/haematol.2023.283295","article-title":"Dabrafenib and Trametinib in Langerhans Cell Histiocytosis and Other Histiocytic Disorders","volume":"109","author":"Cournoyer E.","year":"2024","journal-title":"Haematologica"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.14510"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000002394"},{"issue":"4","key":"e_1_2_8_27_1","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1007\/s12149-007-0110-3","article-title":"Erdheim\u2010Chester Disease: A Rare Syndrome With a Characteristic Bone Scintigraphy Pattern","volume":"22","author":"Spyridonidis T. J.","year":"2008","journal-title":"Annals of Nuclear Medicine"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0560.2010.01650.x"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-05-430140"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1158\/1535-7163.MCT-18-1244"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.8.22.2770"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature10630"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature10539"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.20865"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-020-74237-z"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2018.05.001"}],"container-title":["Journal of Cutaneous Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cup.70229","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/cup.70229","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cup.70229","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:35:51Z","timestamp":1790768151000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/cup.70229"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,30]]},"references-count":35,"alternative-id":["10.1111\/cup.70229"],"URL":"https:\/\/doi.org\/10.1111\/cup.70229","archive":["Portico"],"relation":{},"ISSN":["0303-6987","1600-0560"],"issn-type":[{"value":"0303-6987","type":"print"},{"value":"1600-0560","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,30]]},"assertion":[{"value":"2026-04-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-25","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-30","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"cup.70229"}}