Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can involve the airway. Recent studies have shown their malignant behavior with local recurrence and potential metastatic spread; half of the cases are associated with anaplastic lymphoma kinase gene rearrangement. Complete surgical resection is recommended, when feasible. We present a case of a 26-year-old woman admitted to our institution with severe respiratory failure; she was affected with primary IMT of the trachea and underwent complete surgical resection.
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