ArticleFrontiers in medicine2026
Primary pulmonary mesenchymal malignancy associated with paraneoplastic pemphigus: a case report.
Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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6 authors.
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Abstract
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.
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