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Glomus Tumor of the Trachea: Value of Multidetector Computed Tomographic Virtual Bronchoscopy

2004; Elsevier BV; Volume: 79; Issue: 2 Linguagem: Inglês

10.4065/79.2.237

1942-5546

Hassan F. Nadrous, Mark S. Allen, Brian J. Bartholmai, Gregory L. Aughenbaugh, Jason S. Lewis, James R. Jett,

Myasthenia Gravis and Thymoma

Glomus tumor of the trachea is extremely rare. We report a case of tracheal glomus tumor in a 39-year-old man who presented with hemoptysis. The diagnosis was made after bronchoscopic biopsy of a tumor involving the posterior wall of the upper trachea. Thin-section multidetector computed tomography of the chest was performed before surgical resection, with multiplanar re-formations and 3-dimensional virtual bronchoscopic reconstruction. Tracheal sleeve resection with reconstruction was successful, and pathological studies confirmed complete resection and the diagnosis of glomus tumor. The patient was disease-free 3 months postoperatively. To our knowledge, this is the first reported case in which additional computed postprocessing was used to help evaluate the extent of such a tumor. Glomus tumor of the trachea is extremely rare. We report a case of tracheal glomus tumor in a 39-year-old man who presented with hemoptysis. The diagnosis was made after bronchoscopic biopsy of a tumor involving the posterior wall of the upper trachea. Thin-section multidetector computed tomography of the chest was performed before surgical resection, with multiplanar re-formations and 3-dimensional virtual bronchoscopic reconstruction. Tracheal sleeve resection with reconstruction was successful, and pathological studies confirmed complete resection and the diagnosis of glomus tumor. The patient was disease-free 3 months postoperatively. To our knowledge, this is the first reported case in which additional computed postprocessing was used to help evaluate the extent of such a tumor. Glomus tumors are composed of modified smooth muscle cells that are similar to those of the glomus body, a specialized arteriovenous anastomosis.1Weiss SW Goldblum JR Perivascular tumors.in: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. Mosby, St Louis, Mo2001: 985-1001Google Scholar Although most of these tumors are benign, more aggressive variants have been described.2Folpe AL Fanburg-Smith JC Miettinen M Weiss SW Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors.Am J Surg Pathol. 2001; 25: 1-12Crossref PubMed Scopus (437) Google Scholar Glomus tumors typically involve the skin of the extremities, with the subungual region of the finger being the most common site.1Weiss SW Goldblum JR Perivascular tumors.in: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. Mosby, St Louis, Mo2001: 985-1001Google Scholar It is extremely rare in the tracheobronchial tree—only 15 cases have been reported previously in the literature.3Gowan RT Shamji FM Perkins DG Maziak DE Glomus tumor of the trachea.Ann Thorac Surg. 2001; 72: 598-600Abstract Full Text Full Text PDF PubMed Scopus (36) Google Scholar To our knowledge, this is the first reported case in which 3-dimensional reconstruction of computed tomographic (CT) images was used to visualize the endoluminal component of the tumor and the extraluminal extension of the mass and to examine the remaining tracheobronchial tree. A 39-year-old man was referred to our institution for evaluation of a glomus tumor of the trachea. He had had hemoptysis intermittently for 2½ years before diagnosis of the tracheal tumor. Initially, the hemoptysis was attributed to gastroesophageal reflux with esophageal erosions. The patient underwent esophageal dilation and was treated with a proton pump inhibitor. Seven months before referral, screening CT of the chest showed a tracheal mass. Bronchoscopy revealed a 1.4-cm polypoid tumor arising from the posterior wall of the trachea at the thoracic inlet. The mass was biopsied and diagnosed as a glomus tumor. Presurgical CT of the chest at our institution showed a mass arising from the posterior wall of the trachea 7 cm above the carina (Figure 1). The axial and reformatted images showed a 1.2 × 1.7 × 1.8-cm (anteroposterior, width, and craniocaudal) lobulated mass, and virtual bronchoscopic images clearly demonstrated that the majority of the mass (1.3 × 1.3 × 1.2 cm) was in the tracheal lumen (Figure 2). Pulmonary function testing revealed normal flow-volume curves. Via a collar incision, the patient had complete resection of the tumor and a primary tracheal end-to-end anastomosis. Postoperatively, the patient recovered without complications. The surgical procedure confirmed the extraluminal extension of tumor posterolaterally to the left.Figure 2Highly detailed and interactive virtual bronchoscopy can be performed by constructing a 3-dimensional model from very thin computed tomographic sections acquired on a multidetector scanner. This view of the trachea directed from the level of the thoracic inlet demonstrates the posterior lobulated glomus tumor, the degree of airway compromise, and the position of the tumor relative to the carina in the background.View Large Image Figure ViewerDownload (PPT) The resected specimen was a 1.5-cm section of the trachea with a 2.0 × 1.5 × 1.5-cm red-brown polypoid mass arising from the posterior aspect (Figure 3). Microscopically, the tumor was composed of sheets and trabeculae of small monomorphic cells with eosinophilic cytoplasm and round to oval, regular nuclei (Figure 4). The cell aggregates surrounded dilated vascular channels of varying size. The luminal surface was covered by respiratory-type epithelium with foci of squamous metaplasia. Although the tumor was well circumscribed, its deep margin extended through the muscular layers of the membranous trachea to involve the underlying connective tissue. Immunoperoxidase studies performed on paraffinembedded sections of the tumor demonstrated strong and diffuse staining for smooth muscle actin and focal staining for vimentin (Figure 5). The tumor cells were negative for chromogranin. A reticulin stain revealed the presence of numerous reticulin fibers coursing among the tumor cells. Bronchoscopy before the patient was dismissed from the hospital revealed a healed tracheal anastomosis. He was asymptomatic 3 months after surgery.Figure 4Tracheal tumor consists of trabeculae and sheets of uniform cells surrounding dilated vascular channels of varying size (hematoxylin-eosin, original magnification ×40).View Large Image Figure ViewerDownload (PPT)Figure 5Immunoperoxidase stain demonstrates that the tumor cells are strongly positive for smooth muscle actin. Note the round, regular appearance of the nuclei (original magnification ×200).View Large Image Figure ViewerDownload (PPT) Tracheal tumors are rare, and most are malignant.4Mathisen DJ Tracheal tumors.Chest Surg Clin N Am. 1996; 6: 875-898PubMed Google Scholar Endoscopic examination is the most reliable means of establishing the diagnosis and should be performed in patients suspected of having such tumors to confirm the diagnosis and guide management.4Mathisen DJ Tracheal tumors.Chest Surg Clin N Am. 1996; 6: 875-898PubMed Google Scholar First described by Masson5Masson P Le glomus neuromyoarterial des regions táctiles et ses tumeurs.Lyon Chir. 1924; 21: 257-280Google Scholar in 1924, glomus tumors consist of cells that resemble the modified smooth muscle of the normal glomus body.1Weiss SW Goldblum JR Perivascular tumors.in: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. Mosby, St Louis, Mo2001: 985-1001Google Scholar According to the new World Health Organization classification of tumors,6Folpe AL Glomus tumours.in: Fletcher CDM Unni KK Mertens F World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. IARC Press, Lyon, France2002: 136-137Google Scholar they can be divided into 3 broad groups on the basis of their biologic potential: glomus tumors, glomus tumors of uncertain malignant potential, and malignant glomus tumors. The benign form accounts for more than 95% of these lesions, even among referral practices.2Folpe AL Fanburg-Smith JC Miettinen M Weiss SW Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors.Am J Surg Pathol. 2001; 25: 1-12Crossref PubMed Scopus (437) Google Scholar Based on the proportion of glomus cells, vessels, and smooth muscle present, these typical lesions can be further subcategorized as glomus tumor, glomangioma, or glomangiomyoma.6Folpe AL Glomus tumours.in: Fletcher CDM Unni KK Mertens F World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. IARC Press, Lyon, France2002: 136-137Google Scholar7Shin DH Park SS Lee JH Park MH Lee JD Oncocytic glomus tumor of the trachea.Chest. 1990; 98: 1021-1023Crossref PubMed Scopus (39) Google Scholar An oncocytic variant has also been described.7Shin DH Park SS Lee JH Park MH Lee JD Oncocytic glomus tumor of the trachea.Chest. 1990; 98: 1021-1023Crossref PubMed Scopus (39) Google Scholar Although most patients present with subungual involvement, the skin of the hands and forearms are also common sites.1Weiss SW Goldblum JR Perivascular tumors.in: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. Mosby, St Louis, Mo2001: 985-1001Google Scholar8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar Extracutaneous locations are rare and include the vagina, stomach, bone, mediastinum, heart, lung, and trachea.7Shin DH Park SS Lee JH Park MH Lee JD Oncocytic glomus tumor of the trachea.Chest. 1990; 98: 1021-1023Crossref PubMed Scopus (39) Google Scholar Within the respiratory tract, the trachea is the most frequent site. Glomus tumor of the trachea is extremely rare—only 16 cases, including our patient, have been reported in the literature.3Gowan RT Shamji FM Perkins DG Maziak DE Glomus tumor of the trachea.Ann Thorac Surg. 2001; 72: 598-600Abstract Full Text Full Text PDF PubMed Scopus (36) Google Scholar7Shin DH Park SS Lee JH Park MH Lee JD Oncocytic glomus tumor of the trachea.Chest. 1990; 98: 1021-1023Crossref PubMed Scopus (39) Google Scholar, 8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar, 9Arapantoni-Dadioti P Panayiotides J Fatsis M Antypas G Tracheal glomus tumour.Respiration. 1995; 62: 160-162Crossref PubMed Scopus (24) Google Scholar, 10Fabich DR Hafez GR Glomangioma of the trachea.Cancer. 1980; 45: 2337-2341Crossref PubMed Scopus (49) Google Scholar, 11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar, 12Haraguchi S Yamamoto M Nishimura H A glomus tumor of the trachea—a case report [in Japanese].Nippon Kyobu Geka Gakkai Zasshi. 1991; 39: 214-218PubMed Google Scholar, 13Heard BE Dewar A Firmin RK Lennox SC One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy.Thorax. 1982; 37: 97-103Crossref PubMed Scopus (41) Google Scholar, 14Hussarek M Rieder W Glomustumor der Luftröhre.Krebsarzt. 1950; 5: 208-212PubMed Google Scholar, 15Ito H Motohiro K Nomura S Tahara E Glomus tumor of the trachea: immunohistochemical and electron microscopic studies.Pathol Res Pract. 1988; 183: 778-784Crossref PubMed Scopus (24) Google Scholar, 16Kim YI Kim JH Suh JS Ham EK Suh KP Glomus tumor of the trachea: report of a case with ultrastructural observation.Cancer. 1989; 64: 881-886Crossref PubMed Scopus (42) Google Scholar, 17Sheffield E Dewar A Corrin B Addis BJ Conroy B Glomus tumour of the trachea.Histopathology. 1988; 13: 234-236Crossref PubMed Scopus (23) Google Scholar, 18Warter A Vetter JM Morand G Philippe E Tracheal glomus tumour [in French].Arch Anat Cytol Pathol. 1980; 28: 184-190PubMed Google Scholar, 19Watanabe 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: 332-334Crossref PubMed Google Scholar, 20Menaissy YM Gal AA Mansour KA Glomus tumor of the trachea.Ann Thorac Surg. 2000; 70: 295-297Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar The characteristics of these cases are summarized in Table 1. The most common presenting symptoms were hemoptysis, dyspnea, and cough. All patients had tumor arising from the posterior membranous wall of the trachea. None of the tumors was locally invasive or metastasic, but extratracheal extension was seen in 4 cases, including our patient.8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar13Heard BE Dewar A Firmin RK Lennox SC One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy.Thorax. 1982; 37: 97-103Crossref PubMed Scopus (41) Google Scholar Failure to perform surgical resection has reportedly resulted in failure to cure, and bronchoscopic tracheal stenting or laser resection with or without adjuvant radiotherapy should be reserved for patients with inoperable tumors.8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar9Arapantoni-Dadioti P Panayiotides J Fatsis M Antypas G Tracheal glomus tumour.Respiration. 1995; 62: 160-162Crossref PubMed Scopus (24) Google Scholar11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google ScholarTable 1Characteristics of 16 Reported Patients With Glomus Tumor of the Trachea*Values are number of patients unless specified otherwise.Data from references 3 and 7-20.Age range (y)34–74Male:female14:2Symptoms†Some patients had multiple symptoms. Hemoptysis9 Dyspnea8 Cough7 Stridor or hoarseness2 Wheeze2 Chest pain1 None2Tumor size (range) (cm)1.2-4.5Tumor location Posterior wall16 Upper trachea3 Middle trachea7 Lower trachea6Tumor type Classic glomus tumor12 Glomangioma3 Oncocytic glomus tumor1Treatment Surgical resection13 Bronchoscopic resection1 Laser resection with or without radiotherapy2* Values are number of patients unless specified otherwise.† Some patients had multiple symptoms. Open table in a new tab Regardless of site, glomus tumors consist of medium-sized cells with round, regular nuclei and eosinophilic cytoplasm that surround vascular channels of varying size. They are typically smooth muscle actin- and vimentinpositive, with variable staining for desmin.2Folpe AL Fanburg-Smith JC Miettinen M Weiss SW Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors.Am J Surg Pathol. 2001; 25: 1-12Crossref PubMed Scopus (437) Google Scholar3Gowan RT Shamji FM Perkins DG Maziak DE Glomus tumor of the trachea.Ann Thorac Surg. 2001; 72: 598-600Abstract Full Text Full Text PDF PubMed Scopus (36) Google Scholar8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar21Porter PL Bigler SA McNutt M Gown AM The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: an immunohistochemical study and review of the literature.Mod Pathol. 1991; 4: 46-52PubMed Google Scholar Ultra-structural evaluation reveals pinocytotic vesicles and myofibrils with dense bodies within the tumor cells.11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar21Porter PL Bigler SA McNutt M Gown AM The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: an immunohistochemical study and review of the literature.Mod Pathol. 1991; 4: 46-52PubMed Google Scholar The principal differential diagnoses of tracheal glomus tumor include carcinoid and hemangiopericytoma.11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar Like glomus tumors, tracheal carcinoids are rare. They consist of small monomorphic cells arranged in nests, trabeculae, and ribbons. However, the nuclei of tracheal carcinoids have the characteristic appearance of all neuroendocrine tumors, being round and smooth with stippled chromatin. They do not have the prominent vascular channels present within a glomus tumor. Other differentiating features include immunohistochemical positivity for neuroendocrine markers, such as chromogranin and synaptophysin, and the presence of dense-core neurosecretory granules on electron microscopy.22Grogg KL Padmalatha C Leslie KO Bronchial carcinoid tumor with crystalloid cytoplasmic inclusions.Arch Pathol Lab Med. 2002; 126: 93-96PubMed Google Scholar Like glomus tumors, hemangiopericytomas are composed of tumor cells that surround branching capillaries and vascular channels.21Porter PL Bigler SA McNutt M Gown AM The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: an immunohistochemical study and review of the literature.Mod Pathol. 1991; 4: 46-52PubMed Google Scholar However, the vessels within hemangiopericytomas are more often narrow and exhibit a characteristic staghorn appearance. In addition, the tumor cells are often spindleshaped and do not exhibit smooth muscle differentiation, in contrast to glomus tumor cells.21Porter PL Bigler SA McNutt M Gown AM The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: an immunohistochemical study and review of the literature.Mod Pathol. 1991; 4: 46-52PubMed Google Scholar Radiologic differentiation between malignant and benign tracheal tumors is difficult. Features suggestive of benign tumor include a localized mass, size smaller than 2 cm in diameter, a round smooth appearance, and no evidence of ulceration, local invasion, or nodal or distant metastasis.23Stark P Radiology of the Trachea. Thieme Medical Publishers, New York, NY1991: 22-53Google Scholar We report the first case in which thin-section multidetector CT with 3-dimensional reconstruction was used to help evaluate a tracheal glomus tumor. The images were acquired with use of a multidetector scanner (LightSpeed Ultra-16, General Electric, Milwaukee, Wis) utilizing a 1.25-mm reconstruction slice thickness. Multiplanar reformations were performed on an Advantage Windows workstation (Volume Analysis 2, Voxtool version 3.0.5, General Electric), and additional virtual bronchoscopy was performed with use of the InSpace volume rendering software on a Leonardo workstation (Siemens Medical Solutions, Erlangen, Germany). With multidetector CT, multiplanar and 3-dimensional reconstructions can be performed on a dataset containing isotropic voxels. This allows viewing of the pathology with equal resolution in any imaging plane, and detailed volume or surface-rendered virtual bronchoscopic models can be built for interactive analysis of the airways. In particular, this process facilitates accurate assessment of the lesion's surface features, the degree of airway obstruction, and the extraluminal extent of an airway lesion. It also helps to locate the position of the tumor and to determine how much of the trachea will have to be resected for complete excision. Patency of the airways distal to an obstruction can also be assessed better than with fiberoptic bronchoscopy. However, direct fiberoptic visualization continues to have advantages, eg, tissue sampling or therapy can be performed during the bronchoscopic procedure, and sessile mucosal lesions that may not be evident on CT can be visualized. Sleeve resection of the trachea with primary reconstruction is the treatment of choice for glomus tumors.24Grillo HC Mathisen DJ Primary tracheal tumors: treatment and results.Ann Thorac Surg. 1990; 49: 69-77Abstract Full Text PDF PubMed Scopus (347) Google Scholar Bronchoscopic tracheal stenting or laser resection with or without adjuvant radiotherapy should be reserved for patients with inoperable tumors.8Koskinen SK Niemi PT Ekfors TO Sipila J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar9Arapantoni-Dadioti P Panayiotides J Fatsis M Antypas G Tracheal glomus tumour.Respiration. 1995; 62: 160-162Crossref PubMed Scopus (24) Google Scholar11Garcia-Prats MD Sotelo-Rodriguez MT Ballestin 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: 459-464Crossref PubMed Scopus (49) Google Scholar Complete surgical resection is curative and requires no adjuvant treatment.