Abstract
Primary sarcomas of the trachea are rare occurrences that present with nonspecific symptoms, making timely diagnosis difficult. We report a case of primary fibrosarcoma of the trachea that presented with acute airway loss secondary to tracheal discontinuity due to tumor destruction. This unusual clinical presentation highlights the difficulties posed in the diagnosis and management of tracheal sarcomas. A discussion of the relevant literature is presented.
1
Introduction
The vast majority of soft tissue sarcomas originate in the limbs and trunk with only 11% presenting as primary tumors of the head and neck . Sarcomas of the trachea are quite rare and encompass only 3.6% of all primary tracheal tumors. The diagnosis of fibrosarcoma involving the trachea is uncommon and is often a diagnosis of exclusion.
We present here a case of primary fibrosarcoma of the trachea that resulted in acute airway compromise.
2
Case report
The Otolaryngology service was consulted to see a 68 year-old female that was admitted with complaints of persistent shortness of breath and intermittent hoarseness. The patient had a complex medical history including tracheal stenosis after tracheostomy for which she had undergone tracheal dilation 3 months prior to her admission.
The patient’s airway was initially evaluated with flexible fiberoptic laryngoscopy and demonstrated no laryngeal abnormalities with normal true vocal mobility. Over the subsequent 48 h, the patient experienced worsening shortness of breath and was transferred to ICU level care. CT imaging of the neck and chest was scheduled with a plan for intubation prior to transport. However, at the time of attempted transoral intubation, the endotracheal tube could not be passed below the level of the glottis and ENT was emergently called to perform bedside tracheostomy.
After the initial dissection in the lower midline neck, extensive soft tissue necrosis with destruction of the cricoid cartilage and tracheal separation was noted. Following removal of necrotic debris, an endotracheal tube was introduced into the mediastinal aspect of the trachea. The patient remained in stable condition following the procedure despite intermittent air leaks that resulted from the compromised stability of the distal trachea. Subsequent imaging demonstrated marked irregularity and discontinuity of the anterior tracheal wall, as well as non-specific linear hyperdensity along the anterior tracheal margin, but no obvious mass lesions ( Figs. 1, 2 ).
