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A Quite Exceptional Cause of Recurrent Hemoptysis

2013; Elsevier BV; Volume: 144; Issue: 5 Linguagem: Inglês

10.1378/chest.12-1004

1931-3543

Maria‐Rosa Ghigna, Élie Fadel, Roberto Bellini, Adéla Rohnean, Laurent Palazzo, Peter Dorfmüller, Philippe Dartevelle, Vincent de Montpréville,

Salivary Gland Tumors Diagnosis and Treatment

A smoker aged in his 70s was admitted with severe hemoptysis (about 100 mL/24 h). His medical history included systemic arterial hypertension and COPD. The lungs, cardiovascular, abdominal, skin, and oropharyngeal examinations had normal findings. No cervical adenopathy was detected on physical examination. Chest radiography was performed first but failed to detect the cause of bleeding. Subsequently, the investigation proceedings included CT scan, which revealed a 2-cm-sized lesion that mainly involved the posterior mediastinum and was in close contact with the tracheal wall. After contrast injection, the mass showed a significant enhancement (Figs 1A-C), indicating a solid and vascularized lesion and ruling out the possibility of a fluid collection. Moreover, one enlarged (1.6 cm) left-sided paratracheal node was identified (Fig 1D). Given the size of the lesion, a mediastinal origin could not be excluded; consequently, transesophageal endoscopic ultrasound (EUS) was performed. EUS findings revealed an unremarkable esophageal mucosal surface and a well-circumscribed and vascularized nodule showing mobility with the posterior tracheal wall, thus, suggesting a true tracheal origin (Figs 1E, 1F). A preoperatory endoscopic biopsy was performed after arterial embolization through femoral artery access to minimize the risk of bleeding. The bronchoscopic examination found a bulging erosion of the posterior tracheal wall and a biopsy specimen was taken. Unfortunately, the specimen was too superficial and not helpful for diagnostic purposes. The patient underwent mediastinoscopy and segmental tracheal resection. In the first instance, the frozen section of one enlarged left-sided tracheal node did not reveal malignant disease; in the second, the intraoperative histologic examination of the mass on surgical specimen permitted diagnosis, which was confirmed by paraffin-embedded formalin-fixed tissue samples. The histologic features were similar to the findings of case 2. A male smoker aged in his 40s was admitted to elucidate recurrent episodes of severe hemoptysis (about 100 mL/24 h). His medical history included an intracranial meningioma presenting with seizures that was surgically excised in 1992 without complications. The physical examination was unremarkable. The bronchoscopic examination, which was performed shortly after chest radiography, found a 1-cm-sized tumor bulging into tracheal lumen and located in the posterior tracheal wall 7.5 cm distal to the vocal cords and 3 cm proximal to the carina. A biopsy of the tumor, with unknown bleeding consequences, was performed. Chest radiography did not show any thoracic lesions. The CT scan was performed after endoscopy because it was not available at the time of patient admission. It showed a well-delimited tracheal nodule unequivocally located in the posterior wall. Contrast CT scan showed similar imaging features as those described in patient case 1 (a well-circumscribed, solid, and hypervascularized nodule). Neither mediastinal involvement nor adenopathies were found. Pulmonary fields were unremarkable. The histologic features comprised a monotonous proliferation of medium-sized cells with a distinct cellular border arranged in lobules, nests, or cords and spaced by a dense vascular meshwork (Fig 2). No mitotic figures or necrotic foci were observed. Immunohistochemistry showed labeling for smooth muscle actin, whereas no staining was found for chromogranin A. The patient underwent a segmental resection of the trachea with an end-to-end anastomosis. The postoperative course was uneventful. The surgical specimen measured 1.4 cm in length with a gray-pink, rubbery, transmural, 1-cm mass in its center. The surface of the mass was covered by a focally eroded hyperemic tracheal mucosa. The solid and well-circumscribed lesion was localized in the right posterior angle. Both patients presented with episodes of severe hemoptysis, a potentially life-threatening condition requiring well-timed multidisciplinary management. Diagnostic means to investigate hemoptysis include chest radiography, bronchoscopy, and CT imaging. At present, no consensus guidelines exist about an optimal diagnostic approach to hemoptysis. Some reports highlighted the effectiveness and safety of high-resolution CT scan as the first-line tool for identifying the cause of bleeding (especially in cases of bronchiectasis-related hemoptysis).1Sakr L Dutau H Massive hemoptysis: an update on the role of bronchoscopy in diagnosis and management.Respiration. 2010; 80: 38-58Crossref PubMed Scopus (168) Google Scholar, 2Revel MP Fournier LS Hennebicque AS et al.Can CT replace bronchoscopy in the detection of the site and cause of bleeding in patients with large or massive hemoptysis?.AJR Am J Roentgenol. 2002; 179: 1217-1224Crossref PubMed Scopus (117) Google Scholar, 3Khalil A Soussan M Mangiapan G Fartoukh M Parrot A Carette MF Utility of high-resolution chest CT scan in the emergency management of haemoptysis in the intensive care unit: severity, localization and aetiology.Br J Radiol. 2007; 80: 21-25Crossref PubMed Scopus (60) Google Scholar Nevertheless, others have supported it as a complementary investigation to fiber-optic bronchoscopy for bleeding site detection.2Revel MP Fournier LS Hennebicque AS et al.Can CT replace bronchoscopy in the detection of the site and cause of bleeding in patients with large or massive hemoptysis?.AJR Am J Roentgenol. 2002; 179: 1217-1224Crossref PubMed Scopus (117) Google Scholar In both patients, chest radiography was performed as the first-line examination to investigate hemoptysis, but it was noncontributive. The next step in the diagnostic process in patient case 1 was CT scan, whereas in patient case 2, bronchoscopy was carried out first because it was more readily available than CT imaging at the time of admission. The endoscopic findings were similar in both cases and agreed with those reported in literature.4Kim 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, 5Gowan 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, 6Lange TH Magee MJ Boley TM Bell SW Hazelrigg SR Tracheobronchial glomus tumor.Ann Thorac Surg. 2000; 70: 292-295Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar, 7Menaissy 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, 8Shang Y Huang Y Huang HD et al.Removal of glomus tumor in the lower tracheal segment with a flexible bronchoscope: report of two cases.Intern Med. 2010; 49: 865-869Crossref PubMed Scopus (16) Google Scholar, 9Parker KL Zervos MD Donington JS Shukla PS Bizekis CS Tracheal glomangioma in a patient with asthma and chest pain.J Clin Oncol. 2010; 28: e9-e10Crossref PubMed Scopus (8) Google Scholar Glomus tumor endoscopic findings are distinctive in all cases described. The tumors, sized between 1 and 2.5 cm in largest diameter, developed mainly in the middle or lower tracheal third and presented as a polypoid mass systemically arising from the posterior wall. Respiratory mucosa covering the tumoral mass appeared fragile and hyperemic or focally ulcerated. Glomus tumor appears as a well-delimited mass that partially obstructs airways10Park CM Goo JM Lee HJ Kim MA Lee CH Kang MJ Tumors in the tracheobronchial tree: CT and FDG PET features.Radiographics. 2009; 29: 55-71Crossref PubMed Scopus (101) Google Scholar and is clinically characterized by hemoptysis and wheezing. In both patients, chest radiography was performed but did not detect the cause of bleeding. CT scan (with and without contrast injection) provided major clues to characterize the cause of hemoptysis, establishing the diagnosis of a highly vascular neoplastic process and restricting the spectrum of presurgical diagnostic hypotheses, including neuroendocrine tumor (carcinoid), paraganglioma, soft tissue tumor (eg, endobronchial schwannoma, glomus tumor, angioleiomyoma), and lymphoma. In addition, CT imaging features ruled out the hypothesis of a bronchogenic cyst. The CT image findings of a well-circumscribed, solid, and vascularized lesion agreed with those of tracheal glomus tumors presented in previous cases reports. Lange et al6Lange TH Magee MJ Boley TM Bell SW Hazelrigg SR Tracheobronchial glomus tumor.Ann Thorac Surg. 2000; 70: 292-295Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar reported that the tracheal glomus tumors were all located in the posterior wall and more frequently found in the lower third (seven of 13 cases), followed by the middle third (four cases) and the upper third (two cases). Although in patient case 2 the CT scan revealed that the tumor originated from the tracheal wall, the interpretation of the imaging in patient case 1 proved more difficult because of the greater size of the lesion; hence, mediastinal origin could not be ruled out. An important contribution to the diagnostic process in patient case 1 came from the transesophageal EUS. The EUS showed a well-circumscribed nodular lesion forcing back the esophagus and mobile with the tracheal wall but not with mediastinal tissues. This finding finally supported tracheal origin. Esophageal or endobronchial EUS could be a diagnostic means to investigate lesional features and adenopathies. By reason of their vascularization, biopsy is hazardous in these benign tumors, with the risk of uncontrolled bleeding being very high and potentially fatal. In reviewing the literature, we found 17 related case reports4Kim 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, 5Gowan 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, 6Lange TH Magee MJ Boley TM Bell SW Hazelrigg SR Tracheobronchial glomus tumor.Ann Thorac Surg. 2000; 70: 292-295Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar, 7Menaissy 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, 8Shang Y Huang Y Huang HD et al.Removal of glomus tumor in the lower tracheal segment with a flexible bronchoscope: report of two cases.Intern Med. 2010; 49: 865-869Crossref PubMed Scopus (16) Google Scholar, 9Parker KL Zervos MD Donington JS Shukla PS Bizekis CS Tracheal glomangioma in a patient with asthma and chest pain.J Clin Oncol. 2010; 28: e9-e10Crossref PubMed Scopus (8) Google Scholar, 11Koskinen SK Niemi PT Ekfors TO Sipilö J Valavaara R Dean PB Glomus tumor of the trachea.Eur Radiol. 1998; 8: 364-366Crossref PubMed Scopus (29) Google Scholar, 12Gombos Z Zhang PJ Glomus tumor.Arch Pathol Lab Med. 2008; 132: 1448-1452PubMed Google Scholar, 13Baek SH Huh DM Park JH Kwak EK Kim BH Han WK Glomangiomyoma of the trachea. Korean.J Thorac Cardiovasc Surg. 2011; 44: 440-443Google Scholar; in no case a preoperatory diagnosis of benign neoplasm was established. Histologic examination of a preoperatory biopsy specimen in the second case and of a frozen section in the first case were useful in establishing the correct diagnosis of glomus tumor. Glomus tumors originate from glomus bodies, which are specialized vascular structures surrounded by nerves mostly disseminated in dermis and functionally involved in temperature balance.12Gombos Z Zhang PJ Glomus tumor.Arch Pathol Lab Med. 2008; 132: 1448-1452PubMed Google Scholar Prominent vascularization is a defining feature of the glomus tumor family, although different categories are identified depending on the relative proportion of vascular channels and smooth muscle. Poor vasculature and a scant smooth muscle component characterize solid glomus tumor. Glomangioma and glomangiomyoma both contain prominent vasculature, with the latter also having a smooth muscle component.12Gombos Z Zhang PJ Glomus tumor.Arch Pathol Lab Med. 2008; 132: 1448-1452PubMed Google Scholar, 13Baek SH Huh DM Park JH Kwak EK Kim BH Han WK Glomangiomyoma of the trachea. Korean.J Thorac Cardiovasc Surg. 2011; 44: 440-443Google Scholar Morphologic features include a trabecular or nested proliferation of monotonous medium-sized cells harboring a dense vascular meshwork. Glomus tumor cells are epithelioid, clear, and actin myofilament rich with round or ovoid nuclei. Glomus tumors usually occur as solitary nodules in glomus body-rich areas, such as the subungual regions or deep dermis of the palm, wrist, forearm, and foot. Deep soft tissues, mediastinum, bones, GI tract, and airways represent uncommon locations. It is worth mentioning that Kim et al4Kim 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 reported some glomus body-like structures in the posterior tracheal wall, thus, providing an explanation of the specific localization of tracheal lesions. Among primary tracheal tumors, the most frequent neoplasms are smoke-related tumors, such as squamous cell carcinoma and small cell carcinoma, and smoke-unrelated lesions, such as adenoid cystic and mucoepidermoid carcinoma14Gaissert HA Grillo HC Shadmehr MB et al.Uncommon primary tracheal tumors.Ann Thorac Surg. 2006; 82: 268-272Abstract Full Text Full Text PDF PubMed Scopus (97) Google Scholar, 15Gaertner EM Steinberg DM Huber M et al.Pulmonary and mediastinal glomus tumors—report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review.Am J Surg Pathol. 2000; 24: 1105-1114Crossref PubMed Scopus (127) Google Scholar, 16Honings J Gaissert HA van der Heijden HFM Verhagen AF Kaanders JH Marres HAM Clinical aspects and treatment of primary tracheal malignancies.Acta Otolaryngol. 2010; 130: 763-772Crossref PubMed Scopus (50) Google Scholar, 17Gaissert HA Mark EJ Tracheobronchial gland tumors.Cancer Contr. 2006; 13: 286-294PubMed Google Scholar (Table 1). Although all the cited neoplasms showed unequivocal morphologic features, carcinoid tumors enter the differential diagnosis with glomus tumor and should be ruled out by immunohistochemistry.12Gombos Z Zhang PJ Glomus tumor.Arch Pathol Lab Med. 2008; 132: 1448-1452PubMed Google Scholar Well-differentiated tracheal neuroendocrine tumors (including carcinoids) are rare smoke-unrelated neoplasms and present as well-demarcated and hypervascularized lesions that are clinically and radiologically similar to glomus tumors. Immunohistochemistry may easily differentiate glomus tumors, which are actin positive, from carcinoids, which express neuroendocrine markers. We believe that immunohistochemistry should be mandatory for the diagnosis of both tumors when located in the posterior tracheal wall.Table 1Tracheal Tumors (Histology, Endoscopic, and CT Scan Findings)Tumor TypeEndoscopic FindingsCT Scan FindingsMalignant Squamous cell carcinoma…Usually solid Salivary gland tumors (including mucoepidermoid carcinoma and adenoid cystic carcinomaaMay appear as well defined on endoscopy and imaging.)…Usually hypervascularized Neuroendocrine tumors (including carcinoidaMay appear as well defined on endoscopy and imaging.)Bulging, circumferential, or plaque-like lesionPartially necrotic Sarcoma Hematolymphoid malignanciesUlcerationInfiltrative Metastasis…Adjacent organ invasion Malignancies directly invading tracheal wall (thyroid carcinoma, laryngeal carcinoma, esophageal carcinoma, hematolymphoid malignancies, soft tissue tumors)…Often associated with metastatic nodesBenign Squamous cell papillomaNodular or polypoidWell defined Granular cell tumor…… Schwannoma…… ParagangliomaWell circumscribedMay be hypervascularized LipomaMucosal erosionAbsence of adjacent organ invasion Glomus tumor……a May appear as well defined on endoscopy and imaging. Open table in a new tab Because glomus tumors are almost exclusively benign, the treatment is complete surgical resection without nodal dissection.16Honings J Gaissert HA van der Heijden HFM Verhagen AF Kaanders JH Marres HAM Clinical aspects and treatment of primary tracheal malignancies.Acta Otolaryngol. 2010; 130: 763-772Crossref PubMed Scopus (50) Google Scholar Present patients underwent segmental tracheal resection with an unremarkable clinical course and no recurrence. Similar results are reported after surgery in 14 cases described in the literature.6Lange TH Magee MJ Boley TM Bell SW Hazelrigg SR Tracheobronchial glomus tumor.Ann Thorac Surg. 2000; 70: 292-295Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar Endoscopic ablation has been undertaken in a few cases, but because glomus tumors frequently extend into the entire tracheal wall, this therapeutic option is not recommended.8Shang Y Huang Y Huang HD et al.Removal of glomus tumor in the lower tracheal segment with a flexible bronchoscope: report of two cases.Intern Med. 2010; 49: 865-869Crossref PubMed Scopus (16) Google Scholar Tracheal glomus tumor is a rare, almost always benign neoplasm with some characteristic features, such as location (posterior tracheal wall), and clinical presentation and should be considered in the presurgical diagnostic workup of tracheal tumors.