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CORRESPONDENCE
Year : 2013  |  Volume : 9  |  Issue : 2  |  Page : 302-304

Primary adenoid cystic carcinoma of lung: A case report and review of the literature


Department of Radiotherapy and Oncology, Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication13-Jun-2013

Correspondence Address:
Rakesh Kapoor
Department of Radiotherapy, Post Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.113399

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 > Abstract 

Primary adenoid cystic carcinoma of lung is an unusual thoracic neoplasm, which is considered as a slow-growing low-grade malignancy. The primary treatment for this tumor is surgery. The role of radiotherapy, chemotherapy, and targeted agents is less well defined. Here we report a case of inoperable adenoid cystic carcinoma of the lung in a 42-year-old male, presenting with an unusual aggressive behavior. The patient received radiotherapy and oral tyrosine kinase inhibitor imatinib with effective palliation.

Keywords: Adenoid cystic carcinoma, c-kit, imatinib, lung, radiotherapy


How to cite this article:
Bhattacharyya T, Bahl A, Kapoor R, Bal A, Das A, Sharma S C. Primary adenoid cystic carcinoma of lung: A case report and review of the literature. J Can Res Ther 2013;9:302-4

How to cite this URL:
Bhattacharyya T, Bahl A, Kapoor R, Bal A, Das A, Sharma S C. Primary adenoid cystic carcinoma of lung: A case report and review of the literature. J Can Res Ther [serial online] 2013 [cited 2019 Sep 19];9:302-4. Available from: http://www.cancerjournal.net/text.asp?2013/9/2/302/113399


 > Introduction Top


Primary adenoid cystic carcinoma of lung is a rare tumor, and probably accounts for 0.04-0.2% of all primary pulmonary tumors. [1] It was formerly referred to asbronchial adenoma implying a benign glandular neoplasm. However, it is now considered to bea low-grade bronchial carcinoma. [2] Pulmonary adenoid cystic carcinomas (ACC) usually arise in the proximal tracheobronchial tree, and is regarded as a slowly growing tumor. [3]

Although salivary-gland-type tumors generally have good prognosis, adenoid cystic carcinoma may present at a higher stage and are often unresectable or, if resectable, often have positive surgical margins and subsequent local recurrences. [4]

We report a case of alocally aggressive ACC where surgery was not possible.


 > Case Report Top


A 42-year-old man presented with a 2month history of shortness of breath, nonproductive cough,fever on and off, chest pain on the right side, and loss of weight. The patient was a smoker and hadsmoked two packs of cigarettes daily for around 20 years. On clinical examination, the patient was found to be febrile and had severe shortness of breath. On auscultationbreath sounds were diminished in the right chest and crepitation was positive in the lower-right lung zone. There was no lymphadenopathy and rest of the systemic exam was within the normal limit. Patients pulmonary function tests revealed an obstructive lung defect. Chest X-ray showed nonhomogenous opacity in the right mid and lower zone with cystic changes and multiple air fluid levels [Figure 1]. Computed tomography of the chest revealed right-sided centrally located mass with cystic and necrotic changes extending peripherally and invading the pleura and chest wall. There was associated mediastinal lymphadenopathy. It also showed postobstructive pneumonitic changes [Figure 2]. The examined sections of endobronchial lung biopsy that were composed of two fragments showed pseudostratified ciliated columnar epithelium. The subepithelium showed a tumor arranged in glandular and cribriform pattern. Tumor cells showed basaloid features with moderate pleomorphism, round to oval hyperchromatic nucleus [Figure 3]. Features were those of adenoid cystic carcinoma. On immunohistochemical study tumor cells were found to be c-kit positive [Figure 4].
Figure 1: Chest X-ray showing nonhomogenous opacity in the right mid and lower zone with cystic changes and multiple air fluid levels

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Figure 2: Computed tomography of the chest revealing right-sided centrally located mass with cystic and necrotic changes extending peripherally and invading pleura and chest wall and was also associated with mediastinal lymphadenopathy

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Figure 3: Photomicrograph showing tumor cells arranged in the cribriform pattern, basaloid cell morphology, and lumen of spaces showing basement membrane like material (H and E, X 200)

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Figure 4: Photomicrograph showing membrane positivity for c-kit in tumor cells (c-kit immunostain, X 200)

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The patient was inoperable due to extensive disease and was referred for radiotherapy. Radiation treatment was given for palliation of chest pain and shortness of breath and effective palliation was achieved with aradiotherapy dose of 20 Gy delivered in five fractionsover 5 days. The patient was tested for c-kit and found to be positive and was treated with Tablet Imatinib 400 mg daily for one year . The patient has completed 1year of follow up after completion of treatment and patient is symptomatically stable with partial response. During chemotherapy patient had developed one episode of skin rashes allover the body, which was managed conservatively.


 > Discussion Top


Adenoid cystic carcinoma (ACC), also known as cylindroma in the past, is a variant of adenocarcinoma with distinct histopathologic and clinical features. ACC occurs most commonly in the salivary glands and, less commonly, at other sites such as the breast, skin, uterine cervix, upper aerodigestive tract, and lung. [4]

Adenoid cystic carcinoma (ACC) is a rare but distinctive salivary gland-type malignant neoplasm that arises infrequently as a primary tumor in the lung. ACC of the lung arises from the tracheobronchial glands distributed in the airway submucosa, with a morphology similar to ACC arising in the salivary glands. The review of the literature shows little information regarding their biological behavior in lung as most of the data comes from single case reports or small series. [5] A correlation between the clinical behavior of ACC and their histological patterns has been suggested. The solid histological pattern has been associated with a more aggressive clinical course and early distant metastases, in contrast to the cribriform typewhich shows a more benign behavior. [6] Howeverin our case, the cribriform subtype-predominant pattern showed a very aggressive clinical course thatis quite unusual for such tumors.Surgical resection appears to be the mainstay of treatment in these tumors. Adjuvant radiotherapy and chemotherapy has been used in a limited number of patients [Table 1]. In a study of 20 patients from Mayo clinic from 1927-1977, Conlan et al. showed superior treatment results in patients with complete surgical resection. [6] Radiation sensitivity is relatively high in these patients implying a potential role for radiotherapy. [9] These tumors are generally not sensitive to chemotherapy but may show partial response totargeted novel therapies.
Table 1: Review of various studies showing characteristics of patients

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KIT expression is commonly detected by immunohistochemistry in adenoid cystic carcinoma of the head and neck. [10] In astudy of 49 patients byAubry et al., primary salivary gland type tumors of the lung diagnosed between 1972 and 2002, were analyzed and 33 patients showed KIT positivity. [11] Hotte et al., [12] reported amulticentric phase II trial of imatinib for patients with recurrent or metastatic ACC strongly over expressingKIT using CD117 immunohistochemistry (50-100% staining in tumor cells). Of six patients availablefor evaluation after 3months of treatment, three patients had stable disease and one patient achieved a partial response. The authors suggested that the benefit of Imatinib may be most pronounced in patients with accelerated tumor growth and strong overexpression of KIT. It is possible that these criteria explain the apparent benefit of Imatinib described in two patients treated by Alcedo et al. [13]


 > Conclusions Top


Adjuvant treatment in adenoid cystic carcinoma of lung is still not well defined. The role of radiotherapy and tyrosine kinase inhibitors like Imatinib needs to be further explored.

 
 > References Top

1.Travis WD, Travis LB, Devesa SS. Lung cancer. Cancer 1995;75:191-202.  Back to cited text no. 1
    
2.Spencer H. Pathology of the lung. 4 th ed. Oxford: Pergamon; 1985. p. 968-9.  Back to cited text no. 2
    
3.Reid JD. Adenoid cystic carcinoma (cylindroma) of the bronchial tree. Cancer 1952;5:685-94.  Back to cited text no. 3
    
4.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. 4
    
5.Lozowski MS, Mishriki Y, Solitare GB. Cytopathologic features of adenoid cysticcarcinoma. Case report and literature review. Acta Cytol 1983;27:317-22.  Back to cited text no. 5
    
6.Ratto GB, Alloisio A, Costa R, Chiaramondia M. Primary peripheral adenoid cystic carcinoma of the lung. A case report. Acta Chir Belg 2003;103:414-5.  Back to cited text no. 6
    
7.Albers E, Lawrie E, Harell JH, Yi ES. Trachebronchial adenoid cystic carcinoma: A clinicopathological study of 14 cases. Chest 2004;125:1160-5.  Back to cited text no. 7
    
8.Mondal A, Saha DK. Primary adenoid cystic carcinoma of lung. A cliniciopathological study. Ind J Thorac Cardiovasc Surg 2008;24:240-3.  Back to cited text no. 8
    
9.Grillo HC, Mathisen DJ. Primary tracheal tumors: Treatment and results. Ann Thorac Surg 1990;49:69-77.  Back to cited text no. 9
    
10.Holst VA, Marshall CE, Moskaluk CA, Frierson HF Jr. KIT protein expression and analysis ofc-kit gene mutation in adenoid cystic carcinoma. Mod Pathol 1999;12:956-60.  Back to cited text no. 10
    
11.Aubry MC, Heinrich MC, Molina J, Lewis JE, Yang P,Cassivi SD, et al. Primary adenoid cystic carcinoma of the lung: Absence of KIT mutations. Cancer 2007;110:2507-10.  Back to cited text no. 11
    
12.Hotte SJ, Winquist EW, Lamont E, MacKenzie M, Vokes E, Chen EX, et al. Imatinib mesylate in patients with adenoid cystic cancers of the salivary glands expressing c-kit: A Princess Margaret Hospital phase II consortium study. J Clin Oncol 2005;23:585-90.  Back to cited text no. 12
    
13.Alcedo JC, Fabrega JM, Arosemena JR, Urrutia A. Imatinib mesylate as treatment for adenoid cystic carcinoma of the salivary glands: Report of 2 successfully treated cases. Head Neck 2004;26:829-31.  Back to cited text no. 13
    


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