|Year : 2013 | Volume
| Issue : 1 | Page : 29-31
Tracheal leiomyoma: A clinical dilemma
Vaishali Gupta1, Rajesh Vishwakarma1, Kalpesh Patel1, Hemina Desai2
1 Department of Otorhinolaryngology , Byramjee Jeejeebhoy Medical College, Civil Hospital, Ahmedabad, Gujarat, India
2 Department of Pathology, Byramjee Jeejeebhoy Medical College, Civil Hospital, Ahmedabad, Gujarat, India
|Date of Web Publication||24-Sep-2013|
Department of Otorhinolaryngology, F 5 ENT Operation Theatre, 2nd Floor, Civil Hospital, Ahmedabad 380 016, Gujarat
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Benign tumors of the trachea are rare with leiomyomas even less common. A high degree of clinical suspicion is required for their diagnosis as they usually mimic chest pathology. We report a case of 23-year-old male who presented with orthopnea since 6 weeks without any improvement despite medical treatment. His chest X-ray was normal but the neck computed tomography scan and flexible bronchoscopy showed a tracheal mass. It was resected by anterior endotracheal approach and diagnosed as leiomyoma on histopathology and immunohistochemistry.
Keywords: Histopathology, immunohistochemistry, tracheal leiomyoma
|How to cite this article:|
Gupta V, Vishwakarma R, Patel K, Desai H. Tracheal leiomyoma: A clinical dilemma. J Laryngol Voice 2013;3:29-31
| Introduction|| |
Tracheal leiomyomas are an uncommon condition with only 43 cases reported in English and Japanese literature.  Dyspnea and wheezing are the most common symptoms of tracheal leiomyoma and have been erroneously ascribed tobronchial asthma. Accurate early diagnosis rests on a high index of clinical suspicion and histological examination of bronchoscopic biopsy specimens or frozen section material.  Recognition of the tracheal leiomyoma is important because of the dramatic relief of symptoms that usually follows surgical removal of the lesion. 
| Case Report|| |
A 23-year-old male was referred from physician to otorhinolaryngologist with complaint of difficulty in breathing since 6 weeks. This difficulty increased on lying down and on exertion. Later, he developed abrupt pauses in speech. There was no cough, hemoptysis, throat pain, chest pain, or fever. Radiological investigation - X-ray soft tissue neck, computed tomography (CT) scan [Figure 1] of neck, and virtual bronchoscopy [Figure 2] showed a soft tissue mass in trachea. The mass was projecting into the trachea from right posterolateral aspect, at the level of thyroid gland anteriorly and C7 to T1 vertebra posteriorly. It was pedunculated and lobular of size 15 × 12 × 12 mm. It was 43 mm below vocal cords and 72 mm above tracheal bifurcation. Flexible fibreoptic bronchoscopy showed a pedunculated mass which moved on breathing. Bronchoscope could not be negotiated beyond the tumor, so excision biopsy was planned.
|Figure 1: Computed tomographyscan of neck showing soft tissue mass in trachea|
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Under sedation horizontal incision was given over midline of neck 5 cm below cricoid cartilage, trachea was exposed and vertical incision was given over trachea. The mass could be seen flapping in the trachea. The margins of incision were retracted and a greyish white lobular soft mass with a thin pedicle at the right posterolateral aspect was seen. The pedicle was cut from its attachment flush to the tracheal wall and cauterized. Mass of size 1.5 × 1.8 cm was delivered [Figure 3]. A cuffed tracheostomy tube was inserted and wound closed. Histopathology showed interlacing bundles of spindle tumor cells, no mitosis, no necrosis, no hemorrhage [Figure 4]. Immunohistochemistry was positive for smooth muscle actin. Hence, it was confirmed as a leiomyoma. Postoperatively patient had no respiratory distress and he was decannulated the next day. On follow-up after 6 months, CT neck and flexible bronchoscopy confirmed no recurrent or residual mass.
|Figure 3: Histopathology shows interlacing bundles of spindle tumor cells, no mitosis, no necrosis, and no hemorrhage|
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| Discussion and Review of Literature|| |
Leiomyoma of respiratory tract is located in the bronchi in 2/3 of patients and in trachea in1/3 patients.  They account for 2% of benign tumors of lower respiratory tract.  Among tracheal neoplasms, leiomyoma is a rare disease.  Majority are located in the distal one-third of trachea, the location, extrathoracic or intrathoracic, dictates the surgical approach to the tumor via neck incision or thoracotomy. 
The tracheal leiomyoma usually arises from smooth muscle cells of the membranous posterior wall of trachea.  These are benign circumscribed firm whitish pink tumors with occasional areas of cystic degeneration due to poor vascularization, usually sessile or pedunculated endophytic obstructive lesions. 
It is mainly noted in men in age ranging from 3 months to 56 years.  One-third of patients present before 20 years, the oldest being reported as 67 years.  Leiomyoma of lung parenchyma is twice as common in females and endobronchial leiomyoma has no sex predilection. 
Recognition and diagnosis of tracheal tumor is important because of dramatic relief of symptoms that follow the surgical removal of the lesion.  Frequently, patients are initially diagnosed as having asthma or chronicbronchitis.  Duration of asthma like symptoms persisted for 9 years before the correct diagnosis was made as reported in one case.  Intermittent or constant dyspnea, orthopnea in absence of significant cardiovascular disease, and paroxysmal attacks of wheezing, precipitated by change in position and showing no response to bronchodilator therapy are the most common complaints occurring due to obstructive nature of the lesion.  Ulceration, secondary infection, and mucosal irritation caused by the lesion results in cough with mucoid, purulent, or bloody sputum. 
Most tracheal neoplasms can be detected on plain radiographs of thoracic inlet, especially in lateral view. Chest radiographs can show sequelae of bronchial obstruction such as atelectasis or pneumonitis.  CT scan of neck and chest assumes a more prominent role in evaluation as it confirms the size and smooth configuration of intratracheal mass, and excludes contiguous mediastinal or parenchymal lung involvement. They appear as a homogenous nodule. 
The absolute diagnosis is dependent on the direct bronchoscopic visualization and pathologic examination.  In general, drug treatment is not effective. Resection of the tumor is the only aim of treatment. To date, several different approaches such as tracheal sleeve resection, carinal resection, endoscopic resection, electrocoagulation, cryotherapy, Nd-YAG laser ablation have been reported. Tracheal sleeve resection has the risk of anastomotic failure such as dehiscence or stenosis. Endoscopic resection and ablation can cause perforation, positive surgical margin, lost removed tumor and occasionally an intratracheal explosion.  One case was reported showing tumors 4 cm above carina, removed by sternotomy and then endotracheal approach.  One case was reported with tumor located in the upper cervical trachea which was approached posteriorly through a lateral cervical incision, thus avoiding need for tracheostomy.  In our case, we advocate that endotracheal approach via anterior tracheal incision is a safe and effective procedure for resection.
Prognosis is excellent after complete resection.  Recurrence is rare with only two cases reported in literature, one which was incompletely removed by bronchoscopy recurred in 7 months, and other was removed by segmental sleeve resection with end-to-end anastomosis which recurred in 12 years.  Calcification in the leiomyoma is rare.  Histopathology confirms the diagnosis. Immunohistochemistry staining for smooth muscle alpha-actin can be done. 
| Conclusion|| |
Being a rare entity and presenting mainly with chest complaints, tracheal leiomyoma is difficult to diagnose. Hence, a patient presenting with long-term chest complaint, should have a soft tissue neck X-ray with X-ray chest, and further a CT scan neck. We should do direct bronchoscpic visualization and resect tumor by any of the various techniques depending upon the location and size. The tumor can be confirmed by histopathology and immunohistochemistry.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]