INTRODUCTION: Tracheal hamartomas are very rare. The diagnosis requires a high index of suspicion.
CASE PRESENTATION: A 40-year-old woman without significant past medical history presented to pulmonary clinic with decreased exercise tolerance, progressively worsening shortness of breath and wheezing for two years. She had a persistant cough productive of white phlegm and “chunky white flecks”. The wheezing had no diurnal variation and had responded to steroids but recurred. Symptoms resulted in multiple visits to emergency rooms and treatments for asthma exacerbations. Two years prior to presentation, pulmonary function tests (PFTs) and laryngoscopic exams were normal. Oxygenation was normal by pulse oximetry. Lung exam revealed normal breath sounds but her forced inspiratory and expiratory effort resulted in intractable coughing. Repeat PFT's now revealed severe obstruction. The flow/volume loop suggested a fixed intrathoracic airway obstruction. Bronchoscopy revealed a large mid-tracheal polypoid mass with a stalk obstructing appoximately 95% of the airway. Computed tomography (CT) confirmed near complete occulusion of the trachea with no other abnormalities. The mass was removed via rigid bronchoscopy. The patient reported complete resolution of symptoms and was discharged home. Histological examination revealed a lesion lined by unremarkable respiratory epithelium. The sub-epithelial region had myxoid stroma with mature adipose tissue and benign cartilaginous tissue. A diagnosis of benign polypoid hamartoma was made.
DISCUSSIONS: Hamartomas are the most common benign tumors of the lung. However, tracheal hamartomas are very rare. Their presence in the trachea can result in severe airway obstruction, often leading to prolonged misdiagnosis of asthma. They are typically composed of cartilage, fat, connective tissue, muscle and incorporated respiratory epithelium. The incidence of pulmonary hamartomas is 0.25% with only 10% of them occurring endobronchially. A review of the literature yields only a few reports of tracheal hamartomas. A 1998 review documented seven cases with tracheal hamartomas, the majority found in men. One series reviewed 27 tracheobronchial tumors over an 18 year period and found only one tracheal hamartoma. A study on argon plasma coagulation for tracheobronchial tumors found 11 patients over a 3 year period; however only one patient with a hamartoma. Little imagery is available to illustrate their obstructive nature. To our knowledge, only one case report shows a bronchofiberscopic image of a tracheal hamartoma. This case is unique in that it yields fiberoptic images of near complete occlusion of the trachea by a hamartoma which are complimented by CT images in a woman.
CONCLUSION: Tracheal hamartomas are benign tumors with potentially severe morbidity. In patients in whom wheezing or asthma does not respond to conventional therapy, one should entertain the diagnosis of tracheal lesions including hamartomas. The treatment for this mimicker of asthma is surgical and reminds us that “not all that wheezes is asthma”.
DISCLOSURE: Adan Mora, Jr., None.