Ann Thorac Surg 2002;74:1704-1706
© 2002 The Society of Thoracic Surgeons
Case report
Endotracheobronchial neurofibromas
Han-Shui Hsu, MD*a*,
Chien-Ying Wang, MDa,
Wing-Yin Li, MDa,
Min-Hsiung Huang, MDa
a Division of Thoracic Surgery, Department of Surgery, Veterans General Hospital—Taipei, National Yang-Ming University, Taipei, Taiwan, People's Republic of China
Accepted for publication June 20, 2002.
* Address reprint requests to Dr Hsu, Division of Thoracic Surgery, Department of Surgery, Veterans General Hospital—Taipei, No 201, Sec 2, Shih-Pai Rd, Taipei, Taiwan, People's Republic of China.
e-mail: hsuhs{at}vghtpe.gov.tw
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Abstract
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Among benign tracheobronchial neoplasms, neurofibromas of neurogenic origin are exceedingly rare. In a search of world literature, only 23 cases of endotracheobronchial neurofibromas in 21 series were found. We report another case of a 52-year-old man who presented to our hospital with the symptoms of intermittent productive cough and fever. Bronchoscopy revealed a round tumor obstructing the lumen of the right main bronchus. Right sleeve pneumonectomy was performed, and neurofibroma was confirmed by pathologic examination. The literature is reviewed.
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Introduction
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Tumors arising from tracheobronchial trees are not common. Most of these tumors are malignant in origin, such as squamous cell carcinoma, adenoid cystic carcinoma, or malignant tumors of thyroid or lung with secondary tracheal or bronchial involvement [1]. Benign tumors of tracheobronchial trees include papilloma, leiomyoma, hemangiomas, and tumors of neurogenic origin [2]. Benign neurogenic tumors originate from the peripheral nervous system, are composed of Schwann cells, and appear as neurilemomas or neurofibromas. Neurilemomas (Schwannomas) are single, encapsulated lesions attached to a nerve but containing no neurites. Neurofibromas are usually multiple, not encapsulated, occur within the nerve sheath, and contain neurites. These two tumors can be found in the mediastinum, lung parenchyma, or within the tracheobronchial trees [3–5]. The incidence of neurofibromas involving the trachea or bronchus is lower than that of neurilemomas. In a search of the world literature, there were only 23 cases of tracheobronchial neurofibromas reported in 21 series. We list the search results and present the clinical details of another patient operated on for a neurofibroma of the right main bronchus.
A 52-year-old man presented to Veterans General Hospital—Taipei with mild shortness of breath. He had experienced intermittent productive cough, repeated respiratory tract infection, and progressive shortness of breath for 1 year. He had a smoking history of a half-pack per day for more than 30 years. Physical examination revealed coarse rales and decreased breathing sounds at the right chest. No evidence of neurofibromatosis or any skin lesion was found. Family history was noncontributory. A pulmonary function test showed moderate restrictive ventilation impairment. The forced excretory volume in 1st second was 2.01 liter. The chest radiography showed abnormal pulmonary infiltration and patchy consolidation in the right lung with volume reduction. Computed tomography demonstrated a homogenous tumor located within the right main bronchus with obstructive pneumonitis of the right lung (Fig 1).
Bronchoscopy revealed a hypervascular, round tumor growth with a wide base arising from the posterior wall of the right main bronchus, about 5 mm distal to the carina, obstructing nearly the whole lumen of the right main bronchus. No biopsy was attempted before operation. On February 22, 2000, the patient underwent right-sided posterolateral exploratory thoracotomy. A 4.0 x 2.5 x 2.0-cm firm, yellowish-brown, well-defined tumor was found at the posteromedial wall of the right main bronchus. The lumen of the right main bronchus was nearly occluded. Consolidation of the right middle and lower lobes was also seen. There was no invasion to adjacent structure. Right sleeve pneumonectomy was performed. The postoperative course was complicated by the anastomotic leakage. The patient survived and was discharged from the hospital in stable condition. Microscopically, the tumor consisted of interlacing, waving, spindle-shaped cells strongly positive for S-100 protein and neural filament positive for Bodian stain, but negative for HHF-35, desmin, and actin immunohistochemically, which confirmed the diagnosis of neurofibroma rather than neoplasms of muscular origin.
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Fig 1. Computed tomography demonstrating a soft tissue mass (arrows) originating from the right main bronchus with right middle lobe involvement.
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Comment
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Benign tumors originating from the tracheobronchial trees are not common. Papilloma, polyps, fibroma, and hamartoma account for most of the benign tumors arising in the tracheobronchial trees, followed by leiomyoma and neurogenic tumors [2]. Neurogenic tumors include neurilemoma (Schwannoma), neurofibroma, and neuroma, which can also be seen in the mediastinum or lung parenchyma. Some can cause external compression of the superior vena cava or bronchus [4]. They rarely occur within the endotracheobronchial trees as primary tumors [5]. Neurilemomas are rarely associated with von Recklinghausen’s neurofibromatosis, rarely undergo malignant change, and often contain degenerative changes. Neurofibromas are frequently associated with von Recklinghausen’s neurofibromatosis, and 12% undergo malignant change. In our review of the literature, we found 14 male and 6 female patients with tracheobronchial neurofibroma, including our presenting case (Table 1).
The ages ranged from 8 to 64 years (mean 33.9). Other than the patients listed in Table 1, Perelman and coworkers reported 2 tracheal neurofibroma cases in their series including 144 tracheal tumors [6]. No clinical detail of these 2 patients was mentioned in the article.
The trachea was the most frequent site involved in the cases of endotracheobronchial neurofibromas. There were 2 cases found to have two lesions during clinical manifestations. One patient had neuofibromas in the right intermedius bronchus and the right main bronchus when presented, while the other patient had the neurofibromas in the right lower lobe bronchus and the larynx removed in different periods of time. It is surprising that no lesion from left-sided bronchial trees was reported. Four out of 18 patients had von Recklinghausen’s neurofibromatosis. The ages of these 4 patients were from 18 to 45 years, average 29 years. Three of 4 patients were male (75%). The clinical symptoms and signs of patients with tracheobronchial neurofibromas were mostly related to airway obstruction resembling patients with tracheal tumors. The initial symptoms presented clinically included cough, wheezing, and dyspnea, which are related to airway obstruction. Repeated respiratory tract infection, hemoptysis, and chest pain are occasionally seen. The procedures for tumor removal consisted of operation, laser vaporization, and bronchoscopic removal. The size of the tumor was recorded in only 5 surgical cases and varied from 1.8 to 6 cm in diameter. There has been no recurrence reported to date.
Patients with tracheobronchial tumors may be asymptomatic for years. Chest roentgenography may reveal infiltration and atelectasis, though the majority of the patients have normal chest films in the initial presentation. Delayed diagnosis is not unusual, as overlying soft tissue obscures the trachea in posteroanterior radiography. Some reported that final diagnosis might be realized after a 10 to 15 month delay from the onset of symptoms [7]. The other diagnostic tools include computed tomography and magnetic resonance imaging, which can demonstrate the size of the tumor and the relationship between the tumor and adjacent tissue. Bronchoscopy may disclose a round, protruded, and sometimes hypervascular tumor. Diagnosis can be obtained through biopsy, though it carries some risk of hemorrhage. Thetreatment for the endotracheobronchial neurofibromas depends on the size and the location of the tumors, and should be conservative. Some small tumors can be removed endoscopically with or without laser. However, if the tumors invade adjacent tissue or result in obstructive pneumonitis, surgery should be undertaken.
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References
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- Weber A.L., Grillo H.C. Tracheal tumors: a radiological, clinical, and pathological evaluation of 84 cases. Radiol Clin North Am 1978;16:227-246.[Medline]
- Miller D.R. Benign tumors of lung and tracheobronchial tree. Ann Thorac Surg 1969;8:542-560.[Medline]
- Dodge T.L., Mahaffey J.E., Thomas J.D. The anesthetic management of a patient with an obstructing intratracheal mass, a case report. Anesth Analg 1977;56:295-298.[Abstract/Free Full Text]
- El Oakley R., Grotte G.J. Progressive tracheal and superior vena caval compression caused by benign neurofibromatosis. Thorax 1994;49:380-381.[Abstract/Free Full Text]
- Horovitz A.G., Khalil K.G., Verani R.R., Guthrie A.M., Cowan D.F. Primary intratracheal neurilemoma. J Thorac Cardiovasc Surg 1983;85:313-320.[Abstract]
- Perelman M.I., Koroleva N., Birjukov J., Goudovsky L. Primary tracheal tumors. Semin Thorac Cardiovasc Surg 1996;8:400-402.[Medline]
- Karlan M.S., Livingston P.A., Baker D.C., Jr Diagnosis of tracheal tumors. Ann Otol 1973;82:790-799.
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Abstract
Introduction
