{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T15:58:48Z","timestamp":1753891128614,"version":"3.41.2"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,5,20]],"date-time":"2025-05-20T00:00:00Z","timestamp":1747699200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Anaplastic lymphoma kinase (<jats:italic>ALK<\/jats:italic>) gene rearrangements have been increasingly detected in mesenchymal neoplasms. <jats:italic>ALK<\/jats:italic>-rearranged mesenchymal neoplasms occur mainly in superficial tissues but rarely in internal organs. Herein, we firstly report a primary lung lesion presenting as a rare <jats:italic>ALK<\/jats:italic>-rearranged mesenchymal neoplasm. The patient diagnosed with primary pulmonary <jats:italic>ALK<\/jats:italic>-rearranged mesenchymal neoplasm (PPAMN) received ensartinib as postoperative adjuvant therapy, achieving a disease-free survival of 10\u202fmonths. Continuation of ensartinib as first-line treatment enabled him to benefit from a partial response, with a progression-free survival of 11\u202fmonths. Second next-generation sequencing (NGS) revealed elevated <jats:italic>HMBOX1::ALK<\/jats:italic> abundance along with secondary <jats:italic>NF2<\/jats:italic> mutation. After local radiotherapy combined with ensartinib continuation, his disease was temporarily stable for 7\u202fmonths. Unfortunately, this disease became uncontrolled with an overall survival (OS) of 34\u202fmonths. This is the first case of <jats:italic>ALK<\/jats:italic>-rearranged mesenchymal neoplasm manifested as a primary lung lesion and a novel <jats:italic>HMBOX1::ALK<\/jats:italic> fusion was identified by NGS. The family of <jats:italic>ALK<\/jats:italic>-rearranged mesenchymal neoplasms is expanding and ensartinib could be a potential treatment option for patients with <jats:italic>HMBOX1::ALK<\/jats:italic>. Repeated biopsy and NGS detection are critical to guide treatment selection at disease progression.<\/jats:p>","DOI":"10.3389\/fmed.2025.1572632","type":"journal-article","created":{"date-parts":[[2025,5,20]],"date-time":"2025-05-20T10:00:12Z","timestamp":1747735212000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review"],"prefix":"10.3389","volume":"12","author":[{"given":"Qi","family":"Liang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xingyu","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ziwei","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yali","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siqi","family":"Ni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tingyu","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiao","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huanhuan","family":"Xu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qixing","family":"Gong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lingxiang","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,5,20]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"4846","DOI":"10.1038\/ncomms5846","article-title":"The 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