{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T06:01:21Z","timestamp":1781071281737,"version":"3.54.1"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T00:00:00Z","timestamp":1781049600000},"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>Primary pulmonary inflammatory myofibroblastic tumor (IMT) associated with paraneoplastic pemphigus (PNP) is extremely rare. IMT is an intermediate (rarely metastasizing) mesenchymal neoplasm rather than a conventional sarcoma. We report a case of a 68-year-old female patient who initially presented with generalized skin rash, oral mucosal ulceration, and fever. Chest CT revealed a thoracic mass (initially difficult to determine whether mediastinal or pulmonary in origin). Subsequent thoracotomy and surgical resection with pathological examination confirmed an ALK-rearranged inflammatory myofibroblastic tumor (SQSTM1: ALK fusion) of pulmonary origin. Oral mucosal pathology, combined with clinical findings, supported a diagnosis of PNP. This case aims to provide reference for the diagnosis and treatment of patients with primary pulmonary IMT complicated by PNP. However, the follow-up period was short and systemic immunosuppressive therapy was not administered; this should be regarded as a cautionary note rather than a therapeutic recommendation.<\/jats:p>","DOI":"10.3389\/fmed.2026.1844671","type":"journal-article","created":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T05:20:52Z","timestamp":1781068852000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary pulmonary mesenchymal malignancy associated with paraneoplastic pemphigus: a case report"],"prefix":"10.3389","volume":"13","author":[{"given":"Yi","family":"Dong","sequence":"first","affiliation":[{"name":"Department of Cardio-Thoracic Surgery, School of Clinical Medicine, North Sichuan Medical College","place":["Nanchong, Sichuan, China"]},{"name":"West China School of Medicine, Sichuan University Affiliated Chengdu Second People\u2019s Hospital, Chengdu Second People\u2019s Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongjie","family":"Guan","sequence":"additional","affiliation":[{"name":"Department of Cardio-Thoracic Surgery, School of Clinical Medicine, North Sichuan Medical College","place":["Nanchong, Sichuan, China"]},{"name":"West China School of Medicine, Sichuan University Affiliated Chengdu Second People\u2019s Hospital, Chengdu Second People\u2019s Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuang","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Cardio-Thoracic Surgery, School of Clinical Medicine, North Sichuan Medical College","place":["Nanchong, Sichuan, China"]},{"name":"West China School of Medicine, Sichuan University Affiliated Chengdu Second People\u2019s Hospital, Chengdu Second People\u2019s Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaowei","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Cardio-Thoracic Surgery, West China School of Medicine, Sichuan University Affiliated Chengdu Second People\u2019s Hospital, Chengdu Second People\u2019s Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiyuan","family":"Ren","sequence":"additional","affiliation":[{"name":"Clinical Medicine, Chengdu Medical College","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ke","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Cardio-Thoracic Surgery, West China School of Medicine, Sichuan University Affiliated Chengdu Second People\u2019s Hospital, Chengdu Second People\u2019s Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,10]]},"reference":[{"key":"ref1","volume-title":"Paraneoplastic Pemphigus","author":"Kappius","year":"2023"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jaad.2023.08.020","article-title":"Paraneoplastic pemphigus\/paraneoplastic autoimmune multiorgan syndrome: part I. 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