{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T09:39:37Z","timestamp":1792143577109,"version":"4.4.0"},"reference-count":28,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,2,26]],"date-time":"2024-02-26T00:00:00Z","timestamp":1708905600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008893","name":"University of Iowa","doi-asserted-by":"publisher","award":["CA97422"],"award-info":[{"award-number":["CA97422"]}],"id":[{"id":"10.13039\/100008893","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Malignant histiocytosis (MH) is an extremely rare neoplasm of the macrophage\u2013dendritic cell lineage. We report the clinical characteristics, molecular aberrations, treatments, and outcomes of patients with MH seen at two referral centers from January 2000 to May 2023. We identified 43 patients with MH, of which 26 had histiocytic sarcoma (MH\u2010H), 9 interdigitating dendritic cell sarcoma (MH\u2010IDC), and 8 Langerhans cell sarcoma (MH\u2010LC). The median age at diagnosis was 61\u2009years (range, 3\u201383). Thirty\u2010three patients (77%) had multifocal disease, while 10 had unifocal involvement. Tumor specimens from 22 patients (51%) underwent targeted next generation sequencing, and 19 of 22 (86%) had at least one pathogenic mutation, including mutations in <jats:italic>MAPK<\/jats:italic> pathway genes (73%). The median overall survival (OS) among the entire cohort was 16\u2009months (95% CI: 8\u201350). The outcomes of those with multifocal disease were significantly shorter than their unifocal counterpart: median OS of 10\u2009months versus 50\u2009months (<jats:italic>p<\/jats:italic>\u2009=\u2009.07). Patients with risk organ involvement (bone marrow, spleen, or liver) had significantly inferior outcomes. Chemotherapy and surgery were the most common first\u2010line treatments for multifocal and unifocal disease, respectively. While the outcome for patients with multifocal disease was poor, there was a subset of patients who had durable responses to treatment. Our study highlights that MH has heterogeneous clinical presentation, frequent oncogenic mutations, and prognosis, which is strongly tied to disease extent and type of organ involvement.<\/jats:p>","DOI":"10.1002\/ajh.27263","type":"journal-article","created":{"date-parts":[[2024,2,27]],"date-time":"2024-02-27T05:19:58Z","timestamp":1709011198000},"page":"871-879","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Clinical characteristics, molecular aberrations, treatments, and outcomes of malignant histiocytosis"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6969-9169","authenticated-orcid":false,"given":"Gordon J.","family":"Ruan","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5074-8453","authenticated-orcid":false,"given":"Saurabh","family":"Zanwar","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4503-440X","authenticated-orcid":false,"given":"Aishwarya","family":"Ravindran","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"},{"name":"Division of Laboratory Medicine\u2010Hematopathology, Department of Pathology The University of Alabama at Birmingham  Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Schram","sequence":"additional","affiliation":[{"name":"Sawtooth Epidemiology &amp; Infectious Diseases  Boise Idaho USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jithma P.","family":"Abeykoon","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2938-3730","authenticated-orcid":false,"given":"Antonious","family":"Hazim","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason R.","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5359-336X","authenticated-orcid":false,"given":"Mithun V.","family":"Shah","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. Nora","family":"Bennani","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liuyan","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Morlote","sequence":"additional","affiliation":[{"name":"Division of Laboratory Medicine\u2010Hematopathology, Department of Pathology The University of Alabama at Birmingham  Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen L.","family":"Rech","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota 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