Primary tracheal hyalinizing clear cell carcinoma

Abstract Hyalinizing clear cell carcinomas (HCCC), now referred to as clear cell carcinomas (CCC) by the World Health Organization (WHO), are rare tumors usually arising from the salivary glands of the head and neck. We present the first case of a CCC originating from the trachea. A 66 year-old woman with history of hypertension, former smoker, presented to the emergency department reporting worsening shortness of breath. Computed chest tomography revealed a polypoid mass arising from the left posterior-lateral wall of the trachea. The patient developed worsening respiratory failure requiring mechanical ventilation. Bronchoscopy was completed which relieved a malignant airway obstruction and pathology revealed nests of cells with monomorphic nuclei and clear cytoplasm set in a hyalinized stroma. Using break apart probe fluorescence in situ hybridization testing the tissue revealed the presence of a recurring translocation of the Ewing sarcoma (EWSR1) gene, confirming the diagnosis. CCC is a rare primary airway tumor. CCC was first reported to originate in the lung in 2015. We present the first case of CCC arising from the trachea.