Journal of Cytology
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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 25  |  Issue : 4  |  Page : 144-146
Primary pulmonary adenoid cystic carcinoma diagnosed by transthoracic fine needle aspiration cytology

Departments of Pathology, TB and Chest Diseases, JLN Medical College, Ajmer, India

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Adenoid cystic carcinoma (ACC) is an uncommon tumor that very rarely affects the lower respiratory tract and lung parenchyma. Fine needle aspiration cytology (FNAC) of primary pulmonary ACC has been rarely described. We report here a 32 year-old female with peripheral ACC, diagnosed by percutaneous transthoracic FNAC. The cytological diagnosis was further confirmed by histological examination of the biopsy specimen.

Keywords: Fine needle aspiration; adenoid cystic carcinoma; lung.

How to cite this article:
Nuwal P, Dixit R, Sharma S. Primary pulmonary adenoid cystic carcinoma diagnosed by transthoracic fine needle aspiration cytology. J Cytol 2008;25:144-6

How to cite this URL:
Nuwal P, Dixit R, Sharma S. Primary pulmonary adenoid cystic carcinoma diagnosed by transthoracic fine needle aspiration cytology. J Cytol [serial online] 2008 [cited 2023 Mar 28];25:144-6. Available from:

   Introduction Top

Adenoid cystic carcinoma (ACC) is a rare but distinctive, salivary gland-type, malignant neoplasm that arises infrequently in the respiratory tract, where it is largely seen in the trachea or large airways. [1] Less commonly (about 10-15%), the pulmonary ACC is seen as a peripheral tumor [2] and often presents as an endobronchial mass lesion causing bronchial obstruction with postobstructive pneumonitis and collapse. Exfoliative cytology is seldom useful in the diagnosis of primary bronchial ACC, because such neoplasms are usually submucosal in location, often with intact mucosa. However, there are a few reports of endobronchial ACC diagnosed on the basis of bronchial brushing cytology also. [3] Fine needle aspiration cytology (FNAC) of primary pulmonary ACC has been rarely mentioned and so far, only one case report describes bronchoscope-guided FNAC of endoscopically visible pulmonary ACC. [4] We present here a case of primary pulmonary ACC diagnosed by percutaneous transthoracic fine needle aspiration, the significance of which is notable in view of the lack of such reports in the literature and the feasibility of this technique in peripheral pulmonary tumors.

   Case Report Top

A 32 year-old female patient presented with a history of right-sided chest pain for the last three months and a single episode of scanty hemoptysis. She denied any history of cough, expectoration, or shortness of breath, fever or any systemic symptoms; her past medical history was also negative. She was a nonsmoker and a housewife.

General physical examination did not reveal any abnormalities. Examination of the respiratory system revealed a dull percussion note, decreased breath sounds, and occasional crackles in the right lower lung fields. The results of other systemic examinations were absolutely normal. Routine investigations of blood and urine were within normal limits. Blood chemistry, electrocardiogram, and ultrasound of the abdomen were also normal. She was human immunodeficiency virus (HIV) seronegative and sputum smear microscopy was negative for acid-fast bacilli. Her chest radiograph showed a homogenous opacity in the right lower zone that was situated posteriorly in the right lateral view. Computed tomography of the thorax could not be done. Fibreoptic bronchoscopy did not reveal any endobronchial abnormality. Bronchial washings were negative for acid-fast bacilli and malignant cells. Transbronchial biopsy was not performed. The patient was subjected to percutaneous transthoracic fine needle aspiration from the lesion under ultrasound guidance. Cytological examination of the aspirates revealed large spherical hyaline globules representing basement membrane material surrounded by neoplastic cells. The cells were cohesive, closely packed, and had uniform round to oval hyperchromatic nuclei with scanty cytoplasm [Figure 1]. These features were suggestive of an adenoid cystic carcinoma.

To confirm the cytological diagnosis, an ultrasound-guided, tru-cut lung biopsy was also performed from the lesion that revealed cribriform pattern of ACC, i.e ., nests and columns of uniform cells arranged concentrically around variably sized, gland-like spaces (pseudocysts). The tumor cells had dense basophilic nuclei without nucleoli, but minimal cytoplasm. The cyst-like spaces were filled with mucoid or hyaline materials which were contiguous with the connective tissue stroma of the tumor [Figure 2]. The patient was referred for surgery to a tertiary hospital.

   Discussion Top

ACCs are slowly growing neoplasms that thicken and narrow the bronchial wall. These are poorly defined, sessile, nodular growths that may ulcerate centrally, but usually infiltrate extensively beneath an intact mucosa, ultimately involving adjacent lung tissue and hilar lymph nodes by direct invasion. Apart from direct extension, these tumors also spread by perineural invasion and the hematogenous route. [5]

Microscopically, ACCs are composed of a rather uniform population of dark-staining, basaloid tumor cells containing a minimal amount of cytoplasm and arranged in three growth patterns: cribriform, solid, and tubular. The cribriform pattern is the most characteristic and recognizable form. It manifests as a punched-out or 'Swiss-Cheese' arrangement of tumor cells, usually surrounding acellular spaces that may contain mucoid or hyaline material. The tumor cells are composed of dense basophilic nuclei containing very inconspicuous or nonexistent nucleoli. Mitotic figures are rarely found. The cell borders are usually indistinct and the nuclear pattern is characteristically angular. The cytological features of this neoplasm mimic those seen in the salivary gland. Typically, the smears show variable cellularity. The monomorphic tumor cells are disposed in syncytial tissue fragments with crowding and overlapping of the hyperchromatic nuclei. The tumor cells show scanty cytoplasm leading to a high nuclear/cytoplasmic ratio. The nuclei are round to oval, with smooth nuclear contours and without prominent nucleoli. The chromatin is usually compact, giving rise to a dark nuclear appearance. A cribriform or sieve-like pattern that encloses spaces of variable size and contains magenta, hyaline globules in air-dried smears is another feature. There may be tight, branching, tubular cluster of cells, imparting a cylindroid or tubular appearance; single cells may be present. The background is usually clean, without any necrosis but extracellular hyaline globules are usually present. [6] Rare cytological findings such as intracellular and extracellular inclusion-like bodies, myxochondroid material, bimorphic populations, and nuclear molding have also been reported, that can cause diagnostic confusion with other lung tumors. [7] The cytological picture in our case was typical of ACC and was further confirmed by histological examination of the biopsy tissue.

The cytopathological differential diagnoses of pulmonary ACCs include reserve cell hyperplasia, carcinoid tumor, small cell carcinoma, well-differentiated adenocarcinoma (primary or secondary) etc in view of small cells and tubular or glandular spaces. [6],[8] In reserve cell hyperplasia, there are compactly arranged, small cells with scanty cytoplasm, round nuclei, and high nuclear/cytoplasmic ratios, that are arranged in sheets with characteristic, attached, columnar cells. Tubular and cribriform structures are also not seen in reserve cell hyperplasia. In carcinoid tumors, small cells are uniform, have rounded nuclei and stippled chromatin with marked cell dissociation. Absence of mucoid globules within glandular structures also differentiate carcinoid tumors from ACCs. In small cell carcinoma, small cells typically show nuclear molding, stippled chromatin, 'traumatic nuclear streaking,' and scanty cytoplasm. The presence of an acellular ball of basement membrane material within the cell clusters favors a diagnosis of ACC over small cell carcinoma. The well-differentiated adenocarcinoma can be ruled out by the presence of a constellation of globular and honeycomb-like arrangement of cells, three-dimensional cell groups with ball-like formations and clusters of uniform cells with cystic spaces containing basement membrane material. [8] The mucinous or basal lamina-like material seen in ACC can be mistaken for several other extracellular substances, such as amyloid, alveolar proteinosis, corpora amylacia, and chondroid material. However, in ACC, the surrounding cells are low-cuboidal and cohesive with characteristic angular hyperchromatic nuclei. [7] The differential diagnosis can also be narrowed by immunohistochemical analysis.

In conclusion, we have presented here a rare case of primary pulmonary ACC that arose as a peripheral lung tumor. The present report also highlights the utility of percutaneous transthoracic FNAC in the diagnosis of primary pulmonary ACC, particularly in peripheral tumors where bronchoscopy may not be very fruitful. The technique is simple, rapid, and cost-effective; however, difficulties might arise when classical features are absent or subtle. In such cases, if adequate quantities of aspirates are obtained, immunohistochemical analysis can be utilized for the diagnosis.

   References Top

1.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.  Back to cited text no. 1  [PUBMED]  
2.Gallagher CG, Stark R, Teskey J, Kryger M. Atypical manifestations of pulmonary adenoid cystic carcinoma. Br J Dis Chest 1986;80:396-9.  Back to cited text no. 2  [PUBMED]  
3.Chuah KL, Lim KH, Koh MS, Tan HW, Yap WM. Diagnosis of adenoid cystic carcinoma of the lung by bronchial brushing: A case report. Acta Cytol 2007;51:563-6.  Back to cited text no. 3  [PUBMED]  
4.Qiu S, Nampoothiri MM, Zaharopoulos P, Logroρo R. Primary pulmonary adenoid cystic carcinoma: Report of a case diagnosed by fine-needle aspiration cytology. Diagn Cytopathol 2004;30:51-6.  Back to cited text no. 4    
5.Fraser RS, Muller NL, Colman N, Pare PD, editors. Fraser and Pare's diagnosis of diseases of the chest. 4th ed. Philadelphia: WB Saunders; 1999. p. 1251-61.  Back to cited text no. 5    
6.Sterrett G, Frost F, Whitaker D. Tumors of lung and mediastinum. In: Gray W, McKee GT, editors. Diagnostic cytopathology. 2nd ed. London: Churchill Livingstone; 2003. p. 97-131.  Back to cited text no. 6    
7.Daneshbod Y, Modjtahedi E, Atefi S, Bedayat GR, Daneshbod K. Exfoliative cytologic findings of primary adenoid cystic carcinoma: A report of 2 cases with a review of the cytologic features. Acta Cytol 2007;51:558-62.  Back to cited text no. 7  [PUBMED]  
8.Lozowski MS, Mishriki Y, Solitare GB. Cytopathologic features of adenoid cystic carcinoma: Case report and literature review. Acta Cytol 1983;27:317-22.  Back to cited text no. 8  [PUBMED]  

Correspondence Address:
Ramakant Dixit
381/26, Ramganj, Ajmer
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9371.50800

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