The Clinical Respiratory Journal LETTER TO THE EDITOR

Glomus tumor of the trachea

Introduction Glomus tumor is a rare vascular tumor exhibiting sheets of smooth muscle and glomus cells on histological examination (1). These tumors mainly involve the nail beds but can also be found in the cerebro-cervical region, gastrointestinal tract, chest wall, mediastinum, respiratory tract and lungs (2). Due to the rarity and wide spectrum of possible presentations in almost all parts of the body, the glomus tumor poses a diagnostic and therapeutic challenge (3). Reviewing the literature, it appears that the endobronchial, glomus tumor mainly involves the trachea. To the best of our knowledge, only four cases of carinal involvement with the lesion have been reported in the English literature (4–7). Here, we report the case of a middle-aged African-American woman, a former smoker with chronic obstructive pulmonary disease (COPD) who was diagnosed with glomus tumor of the carina during investigations for hemoptysis and successfully underwent debulking of tumor through bronchoscopy.

trachea and the right main bronchus (RMB) (Fig. 1B). Brushing and endobronchial biopsies were performed without massive bleeding. In addition, laser ablation as well as Argon plasma coagulation of the mass was performed and near total patency of the trachea and the RMB was established. Tumor debulking was performed successfully without any adverse event. The pathological examination revealed sheets of oval- and spindle-shaped cells surrounded by small blood vessels. The cells had eosinophilic cytoplasm and centrally located nuclei without any evidence of atypia. The cells were positive for smooth muscle actin but negative for keratin AE1/AE3, chromogranin, S-100 and CD34 (Fig. 1C). Although rare, these findings were consistent with glomus tumor arising from the tracheal wall. Following the establishment of the final diagnosis, the patient underwent tumor debulking and was referred to the thoracic surgery group for tumor resection with carinal reconstruction.

Discussion Case report A 50-year-old woman was referred to our clinic with a new onset of hemoptysis of 1-week duration. She also complained of new onset of cough and mild shortness of breath. Her past history was notable for 35 packyear of smoking and cocaine as well as alcohol abuse, kidney stones and a single bout of chronic pancreatitis. She was also diagnosed to have COPD based on her pulmonary function tests. On physical examination, inspiratory wheeze over the lower tracheal region was evident. Her chest X-ray was reported as normal; however, the computed tomography (CT) scan revealed an endobronchial lesion at the level of carina extending into the right main bronchus (Fig. 1A). A flexible bronchoscopy revealed a large friable, exophytic hemorrhagic tumor mass arising from the carina and producing 80+% obstruction of the lower

Conflict of interest The authors have stated explicitly that there are no conflicts of interest in connection with this article.

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2014 John Wiley & Sons Ltd

Glomus tumors are rare, benign tumors accounting for 2% of soft tissue tumors. With respect to their rarity and locations, they may constitute a challenging diagnostic and therapeutic dilemma (1). The most common site of glomus tumors is the nail bed of extremities, which accounts for about 70% of cases. Occasionally, glomus tumors may be found in cerebrocervical region, trunk and gastrointestinal tract (1). In respiratory tract, glomus tumors have been reported in lung parenchyma (2), bronchi (8), carina (5) and trachea. Most of the reported cases of glomus tumors are among middle-aged individuals, yet it can also be found in the adolescent age group. Glomus tumors are more common among men with male to female ratio of 4:1 (5). To date, 25 cases of tracheal (3, 5, 9–14) [reviewed in Mogi et al. (5)] glomus tumors have been reported in which tumor involved carina only in four cases (4–7). With respect to the complexity of the reconstruction of carina, whenever the carina is involved, the patient should be referred to a tertiary center for surgery. Because of rarity of glomus tumors in trachea and particularly carina, they may be misdiagnosed with other pathologies. Differential diagnoses

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Letter to the editor

Aryan et al.

Figure 1. Glomus tumor. (A) Computed tomography image in which the red arrow points to the glomus tumor. (B) Bronchoscopic image of glomus tumor. (C) Pathology of glomus tumor located at mucosal surface; take note of the sheets of smooth muscle cells (×200 magnification).

of tracheal glomus tumor include bronchogenic carcinomas, carcinoid tumors, hemangiopericytoma, smooth muscle neoplasms and neuroectodermal tumors. Bronchoscopic evaluation and biopsy of lesions help establish the correct diagnosis and thereby adequate treatment of patients.

Clinical presentation Most patients with respiratory tract glomus tumor are asymptomatic. When present, dyspnea is the most common presenting symptom; however, patients may also complain from hemoptysis, cough and wheezing (5). In the present case, cough in association with hemoptysis was the main presenting symptom. She also had dyspnea that can be explained as the basis of COPD.

Imaging CT is the best modality to diagnose glomus tumors arising from chest wall, mediastinum, lungs or respiratory tract. Because of its rich vascular nidus, glomus tumors appear as marked enhanced areas in CT images. When originating from the endobronchial tree, glomus tumors may mimic mucosal ball, hemorrhage and more importantly, a malignant process necessitating bronchoscopic evaluations (3, 11, 12, 15). Of note, chest X-ray might be normal in these patients.

Bronchoscopy Glomus tumors usually appear as hemorrhagic, exophitic and friable masses with sessile base. It provides clues to discriminate a glomus tumor from bronchogenic carinomas or metastases. However, the definite diagnosis will be made using pathologic

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examinations of tumor biopsies provided during bronchoscopy (5, 7, 10, 11).

Pathology Glomus tumors are nests of aggregated masses of modified smooth muscle cells arising from a glomus body (1) and hence can be found wherever glomus bodies are present (9). Glomus bodies consist of glomus cells with round- to oval-shaped cells, central nuclei and eosinophilic cytoplasm. With respect to the relative proportion of glomus cells, smooth muscle cells and vascular spaces, glomus tumors are categorized into three subtypes of classic glomus tumors, glomangiomas and glomangiomyomas (1, 7, 10). Visualization of vascular channels and anastomosing nets of smooth muscle cell sheets (positive for smooth muscle actin) strengthens the diagnosis of glomus tumor, particularly when the sample is negative for other markers like CD56 and cytokeratin, which are suggestive of neuroendocrine tumors and bronchogenic carcinomas, respectively (9). A granular cell tumor of carina with eosinophilic cytoplasm, and benign appearance can be considered in differential diagnosis of tracheal glomus tumor. Negative staining for periodic acid–Schiff and S100 help discriminate glomus tumor from granular cell tumor (15).

Treatment and prognosis Non-small cell lung cancer is the most prevalent cause of resectable carinal masses that require sleeve carinal resection and have recurrences with malignant behavior. By contrast, most of the glomus tumors are benign and can be cured with carinal resection and subsequent carinal reconstruction (5). Despite being benign lesions, glomus tumors may behave as aggressive and malignant tumors, (2) and thereby, it seems

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2014 John Wiley & Sons Ltd

Aryan et al.

rational to remove the lesions completely as done for all reported cases to date and the present case. Tumor location can be determined by CT scan and bronchoscopy; however, exact margins should be confirmed by frozen sections during the surgery. Following resection of the tumor, no recurrence has yet been reported, albeit some did not report the follow-up and the others reported short-time follow-up of the patients [ranging from 4 months until 6 years (5)]. In conclusion, glomus tumors are in the differential diagnosis list of tracheal lesions as described in the present case. This is the second report of glomus tumor of trachea in a smoker individual (8). She was a cocaine and an alcohol abuser as well; thus, it is possible that either smoking, cocaine or alcohol or even their combination caused this tumor. Although rare and challenging for diagnosis, glomus tumors are benign lesions that are curable with complete resection. Of note, in case of carinal involvement, the patient should be referred to a tertiary center for reconstruction. Zahra Aryan1,2, Khawaja Salman Zaki1, Michael Machuzak1 and Atul C. Mehta1 1 Cleveland Clinic, Respiratory Institute, Cleveland, OH, USA 2 Student’s Scientific Research Center, Tehran University of Medical Sciences, Tehran, Iran

References 1. Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med. 2008;132(9): 1448–52. 2. Hohenforst-Schmidt W, Woitow M, Zarogoulidis P, et al. Glomus tumor in the lung parenchyma. J Thorac Dis. 2012;4(6): 663–6.

The Clinical Respiratory Journal (2014) • ISSN 1752-6981 © 2014 John Wiley & Sons Ltd

Letter to the editor

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Glomus tumor of the trachea.

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