INTRODUCTION:Granular cell tumor (GCT) is a rare mesenchymal neoplasm, predominantly found in the head and the neck region. The lung is an uncommon site, with around 100 cases reported since first described in the bronchus in 1939. Pulmonary complications from GCT usually present as persistent or recurrent consolidation or atelectasis due to bronchial obstruction. We report a case of endobronchial GCT presenting as hemoptysis.
CASE PRESENTATION:Our patient is a 42 year-old female with 4 pack-year smoking history who was referred for evaluation of 1 month history of cough and hemoptysis. Her review of system was remarkable for 10 pounds weight loss over a one month period, and night sweats without any anorexia or asthenia. Her family history was significant for two sisters with lung cancer .Her physical exam was unremarkable. Laboratory data and Chest X-Ray were within normal limits. Chest CT scan showed a 5 mm non calcified nodule in the left lower lobe with small area of consolidation in the left upper lobe. PPD skin test and 3 sets of sputum were negative for Acid Fast Bacilli. Patient was treated with a course of levofloxacin, but she continued to have recurrent hemoptysis.Flexible bronchoscopy was performed, revealing two submucosal, yellowish nodules in the left upper lobe, at the junction of the apical and posterior segments.Biopsy of these nodules showed the presence of granular cell tumor, with positive immunohistochemical stain for S-100, negative Pancytokeratin stain, and a non contributable CD68. Endobronchial washing from the left upper lobe was negative for acid-fast organisms. Complete endobronchial ablation of both nodules was performed, using argon plasma coagulation. At this point, serial bronchoscopies are planned to monitor for possible recurrence.
DISCUSSIONS:GCTs were first described by Abrikossoff in 1926. They are slow growing tumors that typically present on the tongue, skin, and breast, but may occur in any organ. Involvement of the lung is rare, comprising 6-10% of all GCT, and usually associated with a benign clinical behavior. Histologically, GCT is characterized by a submucosal infiltration of round or oval cells with abundant granular cytoplasm. It is believed that these tumors are of Schwann cell origin. Therefore S-100 protein is usually present in there cells, as seen in our patient. Other immunohistochemical results include positive staining for neuron-specific enolase, Vimentin, Actin, and CD68. Reported cases range from 11 to 84 year old. In one study, there was predominance for upper lobes involvement, as well as involvement of the bronchus intermedius. Tracheal involvement has also been reported. Incidental finding of GCT after imaging or during bronchoscopy is frequent. Centrally located tumors are more likely to cause symptoms including cough, hemoptysis, stridor, dyspnea, with atelectasis and post obstructive pneumonia. It is important to identify the size and the extent to primary tumor as it may affect treatment. Bronchoscopic removal of GCT appears to be adequate for lesions that are less than 8 mm in endoscopic diameter. Tumors with a diameter of 8 mm or greater are likely to invade the full thickness of the bronchial wall, with infiltration of the peribronchial tissue, which can lead to a high recurrence rate (up to 54%). In these cases, a more radical approach such as lobectomy or pneumonectomy may be recommended to avoid recurrence. Surgery is also required for extensive postobstructive lung injury.
CONCLUSION:Granular cell tumors are rare, usually with a benign behavior and tendency to recurrence. Management of these tumors depends on there size, location and extent. Endobronchial ablation using argon plasma coagulation is a safe and effective treatment in non complicated cases. Bronchoscopic follow up may be needed thereafter to detect recurrence.
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