INTRODUCTION: An astute physician is always aware of mimickers of asthma and spares no effort to investigate further at the earliest hint of aberrance. Vocal cord paralysis, foreign body aspiration, tracheomalacia and tumors of the airways are amongst the differentials. We present a case of tracheal adenoid cystic tumor presenting with cough and wheeze.
CASE PRESENTATION: A 45 year old lifelong nonsmoker presented to his primary doctor with a four month history of episodic wheezing and non-productive cough. He was found to have expiratory wheezing on examination and received a short course of antibiotics and bronchodilators. His symptoms improved for several weeks but then recurred with the addition that his cough had become productive of yellow blood streaked sputum. He was diagnosed to have an infective exacerbation of asthma based on his previous presentation and was treated again with bronchodilators and antibiotics. He did not respond and developed worsening cough productive of moderate amounts of bloody sputum. He then came to our hospital for evaluation. Further history revealed that he had travelled to El Salvador one month prior to presentation. He reported no constitutional symptoms. His vital signs were stable. He had no palpable lymphadenopathy and was found to have expiratory wheezing over the upper chest. His chest X-ray showed a widened right paratracheal stripe which on computerized tomogram (CT)appeared to be a mass circumferentially encasing the trachea. Bronchoscopy showed multiple fleshy polypoid lesions three inches above the carina. Endoscopic biopsy confirmed adenoid cystic carcinoma.
DISCUSSION: Primary tracheal tumors are very rare and account for less than 0.5% of all tumors. These tumors are more common in males and most are malignant. The peak incidence occurs in the 5th-6th decades of life. Apart from smoking no other risk factors have been established. Usually insidious in onset, tracheal tumors often present with signs and symptoms of upper airway obstruction. Dyspnea is the most frequent symptom, followed by cough, hemoptysis, wheezing, and stridor. These symptoms are often misinterpreted, leading to a delay in diagnosis. Computerized tomography is the best method of imaging and detecting tracheal or major bronchi lesions. Pulmonary function testing with flow-volume loops may demonstrate flattening of the inspiratory or expiratory loop or both depending on the location. Bronchoscopy is the usual means of obtaining tissue for histologic confirmation and is useful in assessing the resectability of the tumor. The most common histological type of tracheal tumor is squamous cell carcinoma followed by adenoid cystic carcinoma. The remaining cases include a variety of other tumor types including mucoepidermoid carcinoma and other benign tumors. No clear staging system has been established yet. Surgical resection is the treatment of choice for malignant tracheal tumors whenever possible followed by radiation therapy. The most important factor affecting prognosis is the extent of disease at presentation. Those with localized disease have a significantly better prognosis than those with regional disease or distant metastases. Five year survival rates are 47, 26 and 4%, respectively. Patients with squamous cell carcinoma did significantly worse than those with adenoid cystic carcinoma with a five year survival rate of 13 versus 74%.
CONCLUSIONS: Tracheal tumors are rare but due to their insidious onset and similarity in presentation to other common pulmonary conditions there is often a delay in diagnosis. Persistence of symptoms despite treatment and atypical symptoms in an asthmatic should alert the physician to consider other possible diagnoses.
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Reference #2 Urdaneta AI, Yu JB, Wilson LD. Population based cancer registry analysis of primary tracheal carcinoma. Am J Clin Oncol 2011; 34:32.
DISCLOSURE: The following authors have nothing to disclose: Suraj Bopanna, Robert Cohen, Tabassum Nafsi, Abhishek Vedavalli, Kalil Al-Nassir
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