Indian Journal of Pathology and Microbiology

: 2015  |  Volume : 58  |  Issue : 4  |  Page : 516--518

Adenoid cystic carcinoma of the trachea

Heena M Desai1, Rohit Thakare2, Gayathri P Amonkar1, Vinaya Karkhanis2, Jyotsna M Joshi2,  
1 Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India
2 Department of Pulmonary Medicine, TN Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Dr. Heena M Desai
Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Dr. A.L. Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra


Primary salivary gland - type tumors of the lung and airways being unusual, they pose a diagnostic challenge on small biopsies and are usually consigned as non-small cell lung carcinomas. Since the clinical behavior of these tumors is different from the conventional lung tumors, it is important to accurately diagnose them. Among this category of tumors, adenoid cystic carcinoma (ACC) of the lungs and airways is exceedingly rare. Few large studies and maximum case reports have been reported in literature on ACC of the lungs and trachea so far. We herein report a rare case of primary ACC of the trachea diagnosed on bronchoscopic biopsy.

How to cite this article:
Desai HM, Thakare R, Amonkar GP, Karkhanis V, Joshi JM. Adenoid cystic carcinoma of the trachea.Indian J Pathol Microbiol 2015;58:516-518

How to cite this URL:
Desai HM, Thakare R, Amonkar GP, Karkhanis V, Joshi JM. Adenoid cystic carcinoma of the trachea. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Jul 25 ];58:516-518
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Full Text


Primary salivary gland-type tumors of the lung and airways are exceedingly rare accounting for approximately 1% of all pulmonary neoplasms of which primary tracheal tumors comprise just 0.1% of the above. Mucoepidermoid carcinoma is the commonest among them which can involve the trachea followed by adenoid cystic carcinoma (ACC), pleomorphic adenoma, acinic cell carcinoma and epithelial-myoepithelial carcinoma. Since they are rare, they usually are misdiagnosed as other commoner tumors at that site. We report here a case of primary ACC of the trachea and review the literature related to this case.

 Case History

A 42-year-old male presented with complaints of dry cough and dyspnoea on exertion (MMRC Grade II) since 6 months. Spirometry suggested an obstructive abnormality.

Chest X-ray was unremarkable. High resolution computed tomography (HRCT) showed a 3.2 cm × 2 cm isodense mass in the lower trachea extending anteriorly into the right main bronchus suggestive of neoplastic etiology with patchy ground glass opacities noted bilaterally in the lower lobes [Figure 1]. Blood investigations done were within normal limits.{Figure 1}A bronchoscopic biopsy was done which showed normal respiratory lining with underlying tumor arranged in solid nests, cribriform and glandular pattern with many clusters showing central myxoid matrix. The individual tumor cells were compact, polyhedral with a round hyperchromatic nucleus and moderate amount of cytoplasm. Necrosis and hemorrhage were absent. The final histopathological diagnosis was ACC [Figure 2].{Figure 2}

Post-operative recovery was uneventful. The patient was referred to a cancer center for further treatment and was subsequently lost to follow-up.


Primary tracheal tumors are rare. Squamous cell carcinoma and ACC account for 2/3rd of primary tracheal tumors in adults.[1] Since rare, few large studies and maximum case reports have been reported in literature on ACC of the lungs and trachea so far. The largest series done so far was by Maziak et al.[2] who studied 174 patients of tracheal ACC over a 32-year period.

As compared to squamous cell carcinoma of the trachea which predominantly occurs in smokers; ACC is unrelated to smoking.

Symptoms are related to upper airway obstruction and occur only after 50–75% of the luminal diameter is occluded. They are usually misdiagnosed as asthma or chronic obstructive pulmonary disease (COPD). There is no particular sex predilection and usually occurs in the age group of 45–60 years. Our patient was slightly younger.

Computerised tomography (CT) scan is a useful imaging modality in ACC which can help in assessing the tumor location, extraluminal extension, involvement of the carina, right/left main and distal bronchial extension, mediastinal invasion and also distant metastasis.[3]

Also, pulmonary function test (PFT) is obligatory for the diagnosis of tracheal ACC, which shows a fixed airway obstruction with decreased inspiratory and peak expiratory flow.[4]

ACC's are usually centrally located, however can occur peripherally in the lung in occasional cases.[5] They are usually solitary, nodular growths, less than 4 cm in size and have a soft yellow-white cut surface. Histologically, it can have cylindromatous/cribriform, tubular or solid patterns.[6] Purely tubular and solid ACC's are exceedingly rare and most of these tumors show a mixed pattern. All ACC's show a biphasic pattern histologically which can be demonstrated by doing an Alcian Blue-Periodic Acid Schiff (AB-PAS) technique. The stroma/cysts are AB positive whereas the ducts are strongly positive with PAS.[7]

Immunohistochemical stains are not usually required for the diagnosis but can be helpful in certain cases especially to diagnose solid or tubular ACC's. ACC's of the lung show immunohistologic evidence of partial myoepithelial cell differentiation and hence show reactivity for keratin, vimentin, actin, S-100 and CD-117 in some cases.[6]

ACC's are presumably derived from a primitive tracheobronchial gland cell showing differentiation towards ductal and myoepithelial cells.

Various surgical resection options are available depending on the site of airway involvement by ACC. In tracheal ACC, the types of resection can be laryngotracheal, tracheal, and carinal with/without pulmonary resection.[8] Contraindications to surgery include involvement of many lymph nodes, more than 50% tracheal involvement, mediastinal involvement and distant metastasis.[8]

Other treatment modalities include radiotherapy which is usually given as an adjuvant therapy after resection, endotracheal brachytherapy, cisplatin-based chemotherapy, endotracheal debridement and endotracheal stents.[1]

Calzada et al.[9] in a 30 years study of ACC of the airway found that there was a higher disease-free survival rate for patients of airway ACC who underwent surgical resection with adjuvant post-operative radiotherapy. Reports have also shown that the site of airway involvement also plays a role in determining their behaviour; bronchial ACC behaves more aggressively than their tracheal counterparts. Also, patients with an ACC of the distal trachea have a higher risk of local recurrence when compared to proximal tracheal resections. Our patient had a tumor of distal trachea extending to right bronchus anteriorly which could be associated with a poor prognosis.

The 5-year and 10 year survival rate of patients with tracheal ACC ranges from 52% to 79% and 27%–51% respectively; the survival being longer in patients with complete resection, negative airway margins and also in patients who receive adjuvant radiotherapy.[1]

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Conflicts of interest

There are no conflicts of interest.


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