Ann Thorac Surg 2001;72:598-600
© 2001 The Society of Thoracic Surgeons
Case report
Glomus tumor of the trachea
Russell T. Gowan, MDa,b,
Farid M. Shamji, MDa,b,
D. Garth Perkins, MDa,b,
Donna E. Maziak, MDCMa,b
a Division of Thoracic Surgery, University of Ottawa, Ottawa, Ontario, Canada
b Department of Pathology, University of Ottawa, Ottawa, Ontario, Canada
Accepted for publication August 1, 2000.
Address reprint requests to Dr Maziak, Ottawa Hospital Civic Campus, 1053 Carling Ave, CPC Rm. 162, Ottawa, Ontario, Canada, K1Y 4E9
e-mail: dmaziak{at}civich.ottawa.on.ca
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Abstract
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Extracutaneous glomus tumors are uncommon and rarely occur in the trachea. We describe a 73-year-old man with a glomus tumor of the trachea who presented with cough, dyspnea, chest pain, and hemoptysis. A curative segmental tracheal resection with primary reconstruction was performed with no recurrence at 6-year follow-up. The clinicopathologic features of this unusual neoplasm are discussed with a review of the literature.
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Introduction
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Glomus tumors of the trachea are distinctly rare; only 13 cases have been reported in the world literature [1–6]. We describe herein a patient with a tracheal glomus tumor and briefly review the available literature.
A 73-year-old man with longstanding chronic obstructive lung disease, hypertension, and atrial fibrillation presented with a 5-week history of cough, hemoptysis, increasing dyspnea, and retrosternal chest pain relieved by coughing. He had a 12-pack-year history of smoking. On physical examination he had an audible exertional expiratory stridor. Chest roentgenogram was normal. Computed axial tomography (CAT scan) of the neck and thorax revealed a 1-cm polypoid tumor arising from the posterior wall of the lower mid trachea with enlarged lymph nodes in the aortopulmonary window and pretracheal retrocaval space (Fig 1). Rigid bronchoscopy demonstrated a polypoid tumor, with intact overlying mucosa, occupying 60% of the tracheal lumen and extending for 1 cm along the posterior tracheal wall, 8 cm distal to the glottis and 5 cm proximal to the carina. Eighty percent patency of the tracheal lumen was restored by coring out the tumor with biopsy forceps. Pathologic examination demonstrated an incompletely resected glomus tumor.
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Fig 1. Computed tomographic scan of the chest depicting a 1-cm polypoid tumor arising from the posterior wall of the lower mid trachea.
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Two weeks later a 3-cm-long segmental midtracheal resection and primary reconstruction was performed using a transsternal transpericardial incision. It was a complete resection with negative resection margins and normal lymph nodes. The postoperative course was complicated by arrhythmias, pulmonary embolism, and respiratory failure from which the patient fully recovered. At 6-year follow-up there has been no evidence of tumor recurrence.
The surgical specimen consisted of a 3.0-cm segment of trachea containing a nonencapsulated sessile, polypoid, tan-colored 1.6 x 1.3 x 0.6 cm tumor arising from the posterior membranous wall (Fig 2A). Microscopically the completely resected tumor was covered by intact tracheal epithelium with focal squamous metaplasia. It was well circumscribed with pushing margins and was confined within the subepithelial connective tissue and muscular layers. It consisted of anastomosing nests and sheets of very uniform tumor cells that surrounded numerous sinusoidal-type vessels. The tumor cells had eosinophilic cytoplasm and small round nuclei. There was no cellular atypia, necrosis, or mitoses (Fig 2B). Immunohistochemistry showed tumor cells that stained positively for muscle-specific actin and negatively for keratin, factor VIII–related antigen, and neuroendocrine markers. Electron microscopic examination demonstrated cells with features of smooth muscle differentiation, including numerous cytoplasmic bundles of thin fibrils with frequent dense bodies and numerous pinocytotic microvesicles along the plasma membrane. No intracytoplasmic neurosecretory-type, dense core granules were identified.
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Fig 2. (A) Cross-section through the trachea showing the tumor arising from the posterior membranous wall and projecting into the lumen. Scale indicates 1 cm. (B) Photomicrograph of the tumor demonstrating uniform benign cells (arrowhead) and numerous thin-walled blood vessels (arrow). (Hematoxylin-phloxine-raffion stain, x250.)
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Comment
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Glomus tumors originate in the modified smooth muscle cells of the glomus body. With rare exceptions the tumors are benign. Four distinctive subtypes have been classified based on ultrastructural differences and on the relative proportions of glomus cells, vascular structures, and smooth muscle tissue in the tumor. Seventy-five percent are classic glomus tumors, 20% are glomangiomas, and a few cases each of glomangiomyoma and oncocytic glomus tumor have been reported [1]. They most commonly arise in the deep dermis and adjacent subcutaneous tissue, particularly in the subungal region of the finger; other common locations include the palm, wrist, forearm, and foot. Rarely they develop in sites where the normal glomus body is sparse or even absent. Such extracutaneous locations include the stomach, bone, mediastinum, heart, lung, vagina, and trachea [1].
The trachea is an unusual site for both benign and malignant neoplasms, with glomus tumors among the rarest of all benign lesions. Since the initial report by Hussarek and Rieder [7] in 1950 only 14 cases, including the present case, have been described [1–6].
The principal histologic differential diagnosis of glomus tumors includes carcinoids, hemangiopericytomas, and hemangioendotheliomas [2]. These tumors arise in the submucosa of the trachea and consist of sheets and nests of cells surrounding numerous vascular spaces [1]. Glomus tumors have fine reticulin fibers that surround individual small round cells. They exhibit immunostaining to actin and desmin [4]. On electron microscopy there are features of smooth muscle differentiation characterized by a basal lamina envelope, pinocytotic vesicles along the plasma membrane, and intracytoplasmic bundles of myofibrils with electron-dense plaques [4].
In this review of the literature, these 14 patients with glomus tumors of the trachea had an average age of 58 years (range 43 to 74 years), with a male-to-female ratio approximating 4:1. Most were symptomatic presenting with dyspnea, cough, and hemoptysis. Only two patients had signs of acute airway obstruction (Table 1). Unlike their cutaneous counterparts most visceral glomus tumors have been reported as being painless. The patient in this case report was unique in that his glomus tumor caused retrosternal chest pain that was relieved after tracheal resection. All tumors arose from the posterior membranous wall of the trachea and most were in the lower two thirds of the trachea. These neoplasms had an average maximal diameter of 2.0 cm (range 1.2 to 4.5 cm). None of the tumors were locally invasive or metastatic although there were three cases (21%) of extratracheal extension [2, 5]. Even in these cases the pathologic margins of the tumor were pushing as opposed to infiltrative. In all, 71% of patients had a classic glomus tumor, 21% had a glomangioma, and 7% had an oncocytic glomus tumor. This is consistent with the relative incidence rates of glomus tumors at other sites.
Segmental resection of the trachea with primary reconstruction is the treatment of choice for these tumors. Although the follow-up in most reported cases was brief or not stated, the morbidity and recurrence rates appear to be low. For some patients, bronchoscopic resection of the tumor or tracheal stenting was useful in relieving airway obstruction before definitive operation [2]. Given that 21% of patients had extratracheal extension of their tumor bronchoscopic resection, laser, and radiotherapy should be reserved for high-risk patients, as local recurrence may occur [5].
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References
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Shin D.H., Park S.S., Lee J.H., Park M.H., Lee J.D. Oncocytic glomus tumor of the trachea. Chest 1990;98:1021-1023.[Abstract/Free Full Text]
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Garcia-Prats M.D., Sotelo-Rodriguez M.T., Ballestein C., et al. Glomus tumour of the trachea: report of a case with microscopic, ultrastructural and immunohistochemical examination and review of the literature. Histopathology 1991;19:459-464.[Medline]
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Haraguchi S., Yamamoto M., Nishimura H. A glomus tumor of the trachea: a case report. Nippon Kyobu Geka Gakkai Zasshi 1991;39:214-218.[Medline]
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Arapantoni-Dadioti P., Panayiotides J., Fatis M., Antypas G. Tracheal glomus tumour. Respiration 1995;62:160-162.[Medline]
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Koskinen S.K., Niemi P.T., Ekfors T.O., Sipila J., Valavaara R., Dean P.B. Glomus tumor of the trachea. Eur J Radiol 1998;8:364-366.
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Watanabe M., Takagi K., Ono K., et al. Successful resection of a glomus tumor arising from the lower trachea: report of a case. Surg Today 1998;28:332-334.[Medline]
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Hussarek M., Rieder W. Glomustumor der luftrohve. Krabsaret 1950;5:208-212.
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Abstract
Introduction
