{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T03:40:57Z","timestamp":1784259657964,"version":"3.55.0"},"reference-count":26,"publisher":"Harborside Press, LLC","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:p>Survival outcomes of patients with histiocytic neoplasms are poor, with no standard-of-care treatments available for these malignancies. Recent characterization of the genomic landscape of various histiocytic neoplasms have shown a predominance of activating driver mutations within the MAPK\/ERK pathway (ie, <jats:italic>BRAF<\/jats:italic>, <jats:italic>MEK<\/jats:italic>, <jats:italic>KRAS<\/jats:italic>, <jats:italic>MAPK<\/jats:italic>, and <jats:italic>NRAS<\/jats:italic>). Subsequently, successful treatment of these malignancies with BRAF and MEK inhibitors has been reported. This report presents the first patient with histiocytic sarcoma harboring a somatic <jats:italic>KRAS<\/jats:italic> Q61H mutation who was subsequently treated to a near complete response with the MEK inhibitor trametinib. Due to patient preference, lack of standard of care treatments, and associated morbidity from head and neck dissection, initial disease reduction provided by trametinib therapy allowed for a less morbid resection. This case report highlights the utility of up-front next-generation sequencing and the efficacy of MEK inhibition in patients with histiocytic sarcoma harboring activating <jats:italic>KRAS<\/jats:italic> mutations.<\/jats:p>","DOI":"10.6004\/jnccn.2022.7001","type":"journal-article","created":{"date-parts":[[2022,3,25]],"date-time":"2022-03-25T19:13:37Z","timestamp":1648235617000},"page":"618-621","source":"Crossref","is-referenced-by-count":17,"title":["Near Complete Response to Trametinib Treatment in Histiocytic Sarcoma Harboring a Somatic KRAS Mutation"],"prefix":"10.6004","volume":"20","author":[{"given":"Boyu","family":"Hu","sequence":"first","affiliation":[{"name":"1Division of Hematology\/Hematologic Malignancies, Department of Internal Medicine, Huntsman Cancer Institute\/University of Utah, Salt Lake City, Utah;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jay L.","family":"Patel","sequence":"additional","affiliation":[{"name":"2Division of Clinical Pathology, Department of Pathology, ARUP Laboratories and University of Utah, Salt Lake City, Utah;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randa","family":"Tao","sequence":"additional","affiliation":[{"name":"3Department of Radiation Oncology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard B.","family":"Cannon","sequence":"additional","affiliation":[{"name":"4Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Huntsman Cancer Institute\/University of Utah, Salt Lake City, Utah; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus","family":"Monroe","sequence":"additional","affiliation":[{"name":"4Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Huntsman Cancer Institute\/University of Utah, Salt Lake City, Utah; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaurav","family":"Goyal","sequence":"additional","affiliation":[{"name":"5Division of Hematology-Oncology, University of Alabama at Birmingham, Birmingham, Alabama."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"4047","reference":[{"key":"ref241","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1001\/jamaoncol.2017.5029","article-title":"Vemurafenib for BRAF V600-mutant Erdheim-Chester disease and Langerhans cell histiocytosis: analysis of data from the histology-independent, phase 2, open-label VE-BASKET study","volume":"4","author":"Diamond","year":"2018","journal-title":"JAMA Oncol"},{"key":"ref181","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1038\/s41379-020-00673-x","article-title":"The mutational landscape of histiocytic sarcoma associated with lymphoid malignancy","volume":"34","author":"Egan","year":"2021","journal-title":"Mod Pathol"},{"key":"ref131","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1182\/blood-2017-10-812495","article-title":"Histiocytic sarcoma: a population-based analysis of incidence, 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