INTRODUCTION: Sinonasal teratocarcinosarcoma (SNTCS) is a very rare, highly aggressive, rapidly growing tumor that metastasizes to cervical lymph nodes, lungs and the airway. There have been only 63 cases reported to date in the literature. The tumor shows both epithelial and mesenchymal elements including nests of fetal-appearing, non-keratinizing clear cell squamous epithelium. SNTCS affects patients ages 18–79 with an approximate 8:1 male predominance. Three year survival is about 40%. The most common complaints at presentation include nasal obstruction, epistaxis, facial pain, proptosis and visual field deficits. Metastasis to the lungs and airways can cause dyspnea and obstruction of the main bronchi creating challenging situations. We report a case of SNTCS with a very large metastatic mass to the right main bronchus with near-total bronchial obstruction that was completely removed using endoscopic techniques.
CASE PRESENTATION: A 76 year old male with a history of atrial fibrillation, prior stroke and SNTCS first diagnosed in 2003 presented with significant shortness of breath. He had initially been treated with partial resection and radiation therapy. The tumor recurred in 2005 affecting the distal trachea and was treated with laser removal and radiation. He now presented with cough, dyspnea, and weight loss. Admission physical exam showed respiratory distress and decreased breath sounds in the right hemithorax. He had no lymphadenopathy and no visible nasal tumor. CT chest demonstrated a large endobronchial lesion with complete occlusion of right main bronchus. He had no other lesions. He underwent flexible and rigid bronchoscopy. A large glistening mass was noted obstructing the right main bronchus just distal to the carina. Initial biopsy was done and frozen section confirmed metastatic SNTCS. Endobronchial resection was then undertaken. We first circumferentially injected the mucosa around the tumor with dilute epinephrine to achieve hemostasis. The Injection Gold Probe Device, an injection therapy and bipolar electrohemostasis catheter with irrigation capabilities was then used to perform a complete mucosal resection of the tumor. The tumor was too large to be removed through a rigid bronchoscope and had to be removed en-masse with the scope. The patient was re-intubated and inspection of the resection bed showed good hemostasis. Final pathology confirmed this to be a 2.8 × 1.3 cm SNTCS. A second bronchoscopic examination five days later was performed. Additional biopsies from the base of the resected tumor as well as resection margins did not show residual tumor. The patient had a dramatic improvement in his symptoms and was subsequently discharged.
DISCUSSIONS: The cornerstones of treatment for SNTCS include surgical resection as well as radiation therapy with 45–70 Gy. Chemotherapy is rarely effective in this condition. Often, surgical resection is combined with adjuvant radiation therapy, after local resection and debulking have been performed. Options for endobronchial tumor resection include cold biopsy forceps removal, removal with electrocautery and laser destruction. Endobronchial therapy must be individualized depending on tumor size and location.
CONCLUSION: SNTCS is a rare, highly aggressive, rapidly growing tumor. Airway metastasis with bronchial obstruction is a noted complication and efforts should be directed towards symptom palliation. We advocate complete mucosal excision of these tumors, followed by adjuvant radiation therapy.
DISCLOSURE: Alfredo Astua, None.