|Year : 2013 | Volume
| Issue : 2 | Page : 140-142
Tracheal adenoid cystic carcinoma mimicking bronchial asthma
Ashutosh Kansal1, Subhajit Ghosh2
1 Department of Pathology, Institute of Nuclear Medicine and Allied Sciences, Defence Research and Development Organization, New Delhi, India
2 Department of Radiation Biosciences, Institute of Nuclear Medicine and Allied Sciences, Defence Research and Development Organization, New Delhi, India
|Date of Web Publication||4-Jan-2014|
Department of Pathology, Institute of Nuclear Medicine and Allied Sciences, Defence Research and Development Organization, Brig. S. K. Majumdar Road, Timarpur, New Delhi - 110 054
Source of Support: None, Conflict of Interest: None
Adenoid cystic carcinoma (ACC) is a rare primary tracheal malignancy. Hemoptysis, nonproductive cough, dyspnea, chest pain and weight loss are the common initial symptoms. Despite their rarity, their usually insidious onset often leads to a delay in diagnosis, making these potentially treatable lesions difficult to treat and often fatal. We present the case report of a 34-year-old male patient suffering from ACC arising from the right side of the trachea.
Keywords: Adenoid cystic carcinoma, bronchial asthma, trachea
|How to cite this article:|
Kansal A, Ghosh S. Tracheal adenoid cystic carcinoma mimicking bronchial asthma. Indian J Allergy Asthma Immunol 2013;27:140-2
|How to cite this URL:|
Kansal A, Ghosh S. Tracheal adenoid cystic carcinoma mimicking bronchial asthma. Indian J Allergy Asthma Immunol [serial online] 2013 [cited 2020 Feb 27];27:140-2. Available from: http://www.ijaai.in/text.asp?2013/27/2/140/124398
| Introduction|| |
Adenoid cystic carcinoma (ACC) is a rare neoplasm, which usually arises from the salivary, lacrimal or other exocrine glands. It may also arise from the lung, breast, vulva, esophagus, cervix or the external auditory canal tissues. , Second to squamous cell carcinoma, ACC is the most frequent type of neoplasm encountered as primary tracheal tumors.
Tracheal tumors are often misdiagnosed as bronchial asthma and are treated with inhaled steroids and bronchodilators without resolution.  We present a case, in which, the patient had been mistakenly diagnosed with difficult asthma because of the presence of uncontrolled asthmatic symptoms and poor lung functions despite the use of high doses corticosteroids and the mass progressed to cause more than 50% of tracheal obstruction, before it was diagnosed.
| Case Report|| |
This was a case report of a 34-year-old non-smoker man was referred to our hospital with a 2-year history of paroxysmal attacks of dyspnea, noisy breathing and dry cough. He experienced frequent sudden-onset coughing episodes followed by the development of dyspnea a year ago. He was previously diagnosed with difficult asthma and treated with high dose inhaled corticosteroids (1500 μg budesonide) and bronchodilators. Since he was unresponsive to the therapy, he consulted to several institutions to seek medical attention. At the time of admission in our hospital, the patient was asymptomatic. He did not have any strider, wheezing and cyanosis and his vitals were temperature 37°C, respiratory rate 20/min, pulse 82/min and blood pressure 130/70 mmHg. The chest examination was unremarkable. The results of the routine laboratory analysis, including complete blood cell count, chemistry, arterial blood gas, urinalysis and chest X-ray were within the normal limits. On spirometric examination, flow-volume curve display suggested fixed airway obstruction. Forced vital capacity (FVC) was 120% of predicted, forced expiratory volume in 1 s (FEV1) was 30% of predicted and FEV1/FVC was 21%.
During hospitalization, the intermittent spells of respiratory distress continued, interspersed with periods of temporary relief from tracheal suction and oxygen therapy. In order to exclude the possibility of upper airway obstruction, a work-up of computerized tomography (CT) of the chest and fiber optic bronchoscopy (FOB) was obtained. The CT scan illustrated a solid, polyploid intratracheal mass originating from the right side of the trachea at 2 cm proximal of the carina [Figure 1]. FOB revealed a smooth, round mass of 2 cm in diameter originating from the right lateral side of the trachea. The lesion was occupying approximately 50% of the lumen. The lesion was partially excised bronchoscopically in four firm white small pieces, which varied in size from 0.4 to 0.6 cm in greatest dimension and sent for histopathological examination.
|Figure 1: Chest computed tomography scan displaying a polypoid mass occupying almost 50% of the lumen of trachea (white arrow)|
Click here to view
The imprint smear prepared from unfixed tissue revealed clumps of basaloid cells, which were small and oval with scant clear cytoplasm and indistinct cell borders. The nuclei were generally uniform dark and oval without mitosis. Numerous metachromatic hyalinized acellular matrix spheres surrounded by basaloid cells were seen. These features suggested the diagnosis of ACC [Figure 2].
|Figure 2: Photomicrograph of imprint smear showing clumps of basaloid cells and numerous metachromatic hyalinized acellular matrix spheres (arrow head), (MGG ×200)|
Click here to view
The histopathological examination of this lesion showed the tissue to be composed of tubular and cylindrical structures embedded in a loose fibrous connective tissue. The tubular structures were the most prominent and lined by a single layer of epithelial cells containing a lumen in which amorphous faintly pink staining material was present. In some areas, they formed collections of cylindrical structures, which were closely set together and formed a cribriform pattern and in others the tissue grew as solid cords of epithelial cells. The cell nuclei were uniform in size and shape and no mitotic figure was noted [Figure 3]a-c. These findings were interpreted as ACC.
|Figure 3: Photomicrographs of hematoxylin and eosin stained section of excised tissue showing (a) lining of pseudostratiied ciliated columnar respiratory epithelium (arrow) and underlying tumor (arrow head), (×100). (b and c) Uniform epithelial cells arranged in tubular, cylindrical and cribriform pattern in loose connective tissue with intraluminal eosinophilic hyalinized material, (×200)|
Click here to view
| Discussion|| |
The incidence of primary tracheal tumors has been estimated to be 2.7 new cases per million/year affecting men and women with almost equal frequency.  These tumors most frequently occur in the trachea or main stem bronchi and cause symptoms of obstruction, may be misdiagnosed as asthma or bronchitis and the tumor may elude detection for months to years. 
In a review of 44 patients in literature with tracheal ACC, the location was 45.5% in the upper part of the trachea, 20.5% in the middle part of the trachea and 34% in the lower part of the trachea.  A retrospective study of six cases of tracheal ACC, Spizarny et al. found that the sites of tumor were distal trachea in three cases, mid trachea in one case and carina and both main bronchi in one patient; in one patient, the involvement extended from the thoracic inlet to below the origin of both the left and right main bronchi. 
The clinical and pathologic features of ACC of the trachea were initially reported in 1859 by Billroth.  In a study of nine patients, Bagheri et al. found that 88.8% of patients had dyspnea and stridor, 77.7% had cough, 33.3% had bronchospasm similar to asthma and 11.1% had hemoptysis. 
The time from first symptoms to diagnosis varied, ranging from weeks to more than 1 year. Characteristically, ACC is a gradual, low malignancy tumor usually associated with prolonged survival. However, when it is associated with distant metastases survival is frequently <2 years.  ACC spreads most commonly by direct extension, submucosal or perineural invasion, or hematogenous metastasis. More than 50% of patients with tracheal ACC have hematogenous metastases. Pulmonary metastases are the most common and can remain asymptomatic for many years. Regional lymph node metastases are reported in 10% of patients and metastases to the brain, bone, liver, kidney, skin, abdomen and heart have also been reported. ,
Nomori et al. observed that the histological grade of tracheobronchial ACCs correlated with the growth pattern and prognosis. ACCs with tubular/cribriform pattern (grade I) follow an entirely intraluminal growth pattern, while grade III tumors (with >30% solid component) show a predominantly extra luminal growth pattern and metastasis. 
The optimal therapeutic approach is surgical resection and reconstruction. Post-operative irradiation was recommended by most of the authors.  Long-term post-operative follow-up is important to discover recurrences. Despite the malignant features, ACC often follows a prolonged course. Slow and insidious progression, often over several years, is characteristic of even untreated cases. The 5-year survival ranged from 66% to 100% and the 10-year survival ranged from 51% to 62% for patients with tracheal ACC regardless of the treatment. 
| Conclusion|| |
The presenting case report emphasizes the fact that tracheal tumors are uncommon, making up only 0.2% of all respiratory malignancies according to the data from the United States.  The consequence of this low incidence is the lack of meaningful experience with effective therapy. It is critical to bear in mind that if a patient does not respond to appropriate anti-asthma therapy, localized obstructions should be ruled out before establishing the diagnosis of asthma. We need more cooperation across the world to integrate the patient's resources and use the tool of evidence-based medicine to evaluate and formulate the optimized multidisciplinary treatment modality.
| References|| |
|1.||Chin HW, DeMeester T, Chin RY, Boman B. Endobronchial adenoid cystic carcinoma. Chest 1991;100:1464-5. |
|2.||Allen MS Jr, Marsh WL Jr. Lymph node involvement by direct extension in adenoid cystic carcinoma. Absence of classic embolic lymph node metastasis. Cancer 1976;38:2017-21. |
|3.||Azar T, Abdul-Karim FW, Tucker HM. Adenoid cystic carcinoma of the trachea. Laryngoscope 1998;108:1297-300. |
|4.||Mathisen DJ. Tracheal tumors. Chest Surg Clin N Am 1996;6:875-98. |
|5.||Yang PY, Liu MS, Chen CH, Lin CM, Tsao TC. Adenoid cystic carcinoma of the trachea: A report of seven cases and literature review. Chang Gung Med J 2005;28:357-63. |
|6.||Spizarny DL, Shepard JA, McLoud TC, Grillo HC, Dedrick CG. CT of adenoid cystic carcinoma of the trachea. AJR Am J Roentgenol 1986;146:1129-32. |
|7.||Billroth T. Observations on tumors of the salivary glands. Virchows Arch Pathol Anat 1859;17:357-75. |
|8.||Bagheri R, Seyed HFM, Majdi NMA, Rahim MB. Adenoid cystic carcinoma of the trachea. Tanaffos 2008;7:49-54. |
|9.||Moran CA, Suster S, Koss MN. Primary adenoid cystic carcinoma of the lung. A clinicopathologic and immunohistochemical study of 16 cases. Cancer 1994;73:1390-7. |
|10.||Grillo HC. Management of tracheal tumors. Am J Surg 1982;143:697-700. |
|11.||Regnard JF, Fourquier P, Levasseur P. Results and prognostic factors in resections of primary tracheal tumors: A multicenter retrospective study. The French Society of Cardiovascular Surgery. J Thorac Cardiovasc Surg 1996;111:808-13. |
|12.||Nomori H, Kaseda S, Kobayashi K, Ishihara T, Yanai N, Torikata C. Adenoid cystic carcinoma of the trachea and main-stem bronchus. A clinical, histopathologic, and immunohistochemical study. J Thorac Cardiovasc Surg 1988;96:271-7. |
|13.||Grillo HC, Mathisen DJ. Primary tracheal tumors: Treatment and results. Ann Thorac Surg 1990;49:69-77. |
|14.||Wang X, Zhao J, Fang X, Xu N, Zhang X. Adenoid cystic carcinoma, low-differentiation carcinoma, squamous cell carcinoma: A rare evolution of a tracheal malignancy within 27 years. J Clin Oncol 2011;29:e509-11. |
[Figure 1], [Figure 2], [Figure 3]