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Abstract

Case Presentation: A 40-year-old woman with a history of asthma presented with a 2-week history of progressively worsening “heavy” breathing accompanied by an intermittent cough that was productive of blood-tinged sputum. Her physical examination showed wheezing but no stridor. Spirometry testing revealed a forced expiratory volume in 1 second of 1.33 L and a vital capacity of 3.08 L, in comparison with 2.5 L and 3.5 L, respectively, a year previously. The flow volume loop study was significant for flattened inspiratory and expiratory limb curves. Plain radiography of the neck demonstrated a subglottic tracheal mass (Fig. 1, arrow), and reconstructional computed tomographic imaging disclosed a nearly compromised airway (Fig. 2, blue area, arrow). Laryngoscopy revealed a smooth lobulated mass (1.0 by 1.5 cm) that originated from the proximal trachea anteriorly, with an approximated 90% endotracheal narrowing. An emergent resection of a hypervascular tumor was performed, and the patient recovered uneventfully. Immunohistochemical analysis showed that the tumor was strongly positive for neuron-specific enolase, chromogranin (Fig. 3 A), and synaptophysin (Fig. 3 B) and negative for cytokeratin AE1/3 (Fig. 3 C), CAM 5.2, and calcitonin. Sustentacular cells expressed S-100 protein (Fig. 3 D). What is the diagnosis?