{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,24]],"date-time":"2025-12-24T12:37:55Z","timestamp":1766579875479,"version":"3.41.2"},"reference-count":26,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,2,10]],"date-time":"2024-02-10T00:00:00Z","timestamp":1707523200000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Pancreatic acinar cell carcinomas are rare malignant neoplasms. High\u2010quality evidence about the best treatment strategy is lacking. We present the case of a 52\u2010year\u2010old male with a <jats:italic>BRAF<\/jats:italic><jats:sup><jats:italic>V600E<\/jats:italic><\/jats:sup>\u2010mutated PACC who experienced a complete remission after chemotherapy with BRAF\u2010\/MEK\u2010inhibitors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case<\/jats:title><jats:p>The patient presented with upper abdomen pain, night sweat, and weight loss. CT scan showed a pancreatic tumor extending from the pancreas head to body. Histological workup identified an acinar cell carcinoma. As the tumor was inoperable, chemotherapy with FOFIRNIOX was initiated and initially showed a slight regression of disease. The regimen had to be discontinued due to severe side effects. Molecular analysis identified a <jats:italic>BRAF<\/jats:italic><jats:sup><jats:italic>V600E<\/jats:italic><\/jats:sup> mutation, so the patient was started on BRAF\u2010 and MEK\u2010inhibitors (dabrafenib\/trametinib). After 16\u2009months, CT scans showed a near complete remission with a markedly improved overall health.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Studies suggest that up to one\u2010fourth of PACCs carry a <jats:italic>BRAF<\/jats:italic> mutation and might therefore be susceptible to a BRAF\u2010\/MEK\u2010inhibitor therapy. This offers a new therapeutic pathway to treat this rare but malignant neoplasm.<\/jats:p><\/jats:sec>","DOI":"10.1002\/gcc.23222","type":"journal-article","created":{"date-parts":[[2024,2,10]],"date-time":"2024-02-10T12:09:20Z","timestamp":1707566960000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Near complete remission of an inoperable pancreatic acinar cell carcinoma after <scp>BRAF<\/scp>\u2010\/<scp>MEK<\/scp>\u2010inhibitor treatment\u2014A case report and review of the literature"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5296-6355","authenticated-orcid":false,"given":"Lennart","family":"von Fritsch","sequence":"first","affiliation":[{"name":"Department of Surgery University Hospital of Schleswig\u2010Holstein, Campus Luebeck  L\u00fcbeck Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9593-8947","authenticated-orcid":false,"given":"Nikolas","family":"von Bubnoff","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology University Hospital of Schleswig\u2010Holstein, Campus Luebeck  L\u00fcbeck Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Weber","sequence":"additional","affiliation":[{"name":"Luebecker Onkologische Schwerpunktpraxis  L\u00fcbeck Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jutta","family":"Kirfel","sequence":"additional","affiliation":[{"name":"Institute of Pathology, University Hospital Schleswig\u2010Holstein, Campus Luebeck  L\u00fcbeck Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cleopatra","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Institute of Pathology, University Hospital 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