Sameh Aziz

Primary tracheal hyalinizing clear cell carcinoma

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.

Not all right-sided hearts are the same-the importance of identifying the correct diagnosis.

Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CxR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or Scimitar. A 27-year-old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. Her CxR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Chest computed tomography (CT) demonstrated that these opacities join to represent an anomalous vein draining into IVC. Furthermore, an anomalous systemic artery arising from the abdominal aorta was seen to supply the right lower lobe. The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births while nearly half of the patients remain asymptomatic with some initially being misdiagnosed as dextrocardia, such as in our case. Correctly diagnosing these patients is of paramount importance as some can develop severe pulmonary hypertension and right ventricular failure. In turn, close ongoing echocardiographic monitoring can help identify those that may benefit from surgical interventions to prevent them from developing these complications.