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A6745 - Acute Airway Obstruction and Superior Vena Cava Syndrome Secondary to Malignant Teratoma
Author Block: A. Houser, S. Assar, G. T. Chu; University of Arizona College of Medicine, Phoenix, AZ, United States.
Introduction: Intrathoracic germ cell tumors are uncommon, accounting for 1-4% of mediastinal neoplasms. We report a unique presentation of superior vena cava syndrome (SVCS) and acute airway obstruction secondary to a large mediastinal non-seminomatous germ cell tumor (NSGCT), commonly termed a malignant teratoma.
Case: A 47-year-old male with active tobacco use and obesity presented with progressive dyspnea, orthopnea, and face and neck swelling. Three days prior he was treated outpatient with prednisone for suspected tonsillitis. Upon presentation to the emergency department, the patient was in acute respiratory failure secondary to upper airway obstruction and taken for emergent endotracheal intubation and subsequent surgical tracheostomy. Computed tomography of the chest revealed a large anterior mediastinal mass with severe tracheal compression and obstruction of the superior vena cava. Additionally, there were multiple pulmonary nodules, mediastinal adenopathy and a pericardial effusion. Transthoracic biopsy of the mediastinal mass showed well-differentiated fetal elements of all three germ layers consistent with NSGCT. Emergent radiotherapy was performed, and adjunctive corticosteroids were administered. Induction chemotherapy with etoposide and cisplatin was initiated with significant clinical response within 72 hours. Alpha-fetoprotein measurements declined throughout the course of treatment. The patient went on to have resection of residual mediastinal mass and partial pericardiectomy.
Discussion: We present the rare circumstance of a patient presenting with two unique pathologies of a less common mediastinal malignancy. Whereas SVCS is reported in approximately 6% of patients with extragonadal germ cell tumors (GCT), acute airway obstruction as a presenting symptom is sporadic. To our knowledge, this is the first reported case of a simultaneous SVCS and acute airway obstruction secondary to NSGCT. Classic masses arising from the anterior mediastinal compartment include thymoma, lymphoma, teratoma, and normal thyroid tissue. GCT at extragonadal sites are the result of transformation of germ cells which have arrested along the midline urogenital ridge during embryogenesis. The most common site for extragonadal NSGCT is the mediastinum. Extragonadal NSGCT carry a poor prognosis, with five-year survival much lower than primary gonadal tumors. It is important to rule out benign teratoma, lymphoma, and other malignancies. Emergent radiotherapy for SVCS is historically common practice, however newer data suggests deferring treatment until histopathologic diagnosis is established may be appropriate. NSGCT typically responds dramatically to etoposide and cisplatin therapy.
Conclusion: Approximately 86% of patients with malignant GCT are symptomatic upon presentation. Stabilization of life-threatening intrathoracic compression may be complex and require the expertise of multiple specialists.