Primary adenoid cystic carcinoma of the tracheobronchial tree: report of two cases
Ahmed Ben Saad, Rania Kadoussi, Manel Njima, Saousen Cheikh Mhamed, Nesrine Fahem, Nouha Ben Abdeljelil, Samah Joobeur, Naceur Rouatbi
The Pan African Medical Journal. 2019;34:137. doi:10.11604/pamj.2019.34.137.14902

AFRIKA K AFRIKA K
"Better health through knowledge sharing and information dissemination "

Case report

Primary adenoid cystic carcinoma of the tracheobronchial tree: report of two cases

Cite this: The Pan African Medical Journal. 2019;34:137. doi:10.11604/pamj.2019.34.137.14902

Received: 18/01/2018 - Accepted: 25/10/2019 - Published: 08/11/2019

Key words: Adenoid cystic carcinoma, salivary gland cancer, lung cancer, bronchogenic carcinoma

© Ahmed Ben Saad et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Available online at: http://www.panafrican-med-journal.com/content/article/34/137/full

Corresponding author: Ahmed Ben Saad, Pulmonology Department, Fattouma Bourguiba Hospital, Monastir, Tunisia (ahmedbensaad28@yahoo.fr)


Primary adenoid cystic carcinoma of the tracheobronchial tree: report of two cases

Ahmed Ben Saad1,&, Rania Kadoussi1, Manel Njima2, Saousen Cheikh Mhamed1, Nesrine Fahem1, Nouha Ben Abdeljelil2, Samah Joobeur1, Naceur Rouatbi1

 

1Pulmonology Department, Fattouma Bourguiba Hospital, Monastir, Tunisia, 2Department of Pathology, Fattouma Bourguiba Hospital, Monastir, Tunisia

 

 

&Corresponding author
Ahmed Ben Saad, Pulmonology Department, Fattouma Bourguiba Hospital, Monastir, Tunisia

 

 

Abstract

Adenoid cystic carcinoma (ACC) is a rare malignant epithelial tumor that predominantly originates in the salivary glands. Primary ACC of the tracheobronchial tree is extremely rare. We report two new cases of central airways primary ACC: a 58 year-old male with an ACC of the left main bronchus who underwent a pneumonectomy with node dissection, and a 52 year-old female with proximal tracheal ACC presenting as asthma treated by surgical resection and a postoperative radiotherapy. Primary ACC of the tracheobronchial tree is often misdiagnosed given the non-specific clinical presentation. An early diagnosis is essential to ensure good outcomes. An interdisciplinary treatment is required based especially on surgery and radiotherapy.

 

 

Introduction    Down

Adenoid cystic carcinoma (ACC) is a malignant tumor that originate mainly from the salivary glands. The pulmonary localization is rare accounting for 0.04 to 0.2% [1]. It is generally located in the proximal tracheobronchial tree. The involvement of the pulmonary parenchyma is exceptional. Primary pulmonary ACC has no specific clinical or imaging features. The diagnosis is confirmed by histopathology. The evolution is generally favorable provided an early diagnosis and a complete surgical resection [2,3].

 

 

Patient and observation Up    Down

Case 1: a 58 year- old male with 80 pack-years of cigarettes was hospitalized in our department for the exploration of hemoptysis with cough and sputum, initially diagnosed as pneumonia. There is no history of tuberculosis. Chest auscultation revealed ronchi in left basal region. Complete blood count and blood biochemistry were within normal limits. Sputum was negative for acid fast bacilli or other pathogenic germs. His chest X-ray showed a persistent left basal consolidation (Figure 1). Thoracic CT scan revealed a left-sided bronchialcentric mass in the left main bronchus associated with multiple left hilar lymph nodes and carcinomatous lymphangitis in the left inferior lobe (Figure 2). Cerebral and abdomino-pelvic CT scan did not reveal metastatic lesions. Bronchial fibroscopy showed an endobronchial polypoidal growth in the left main bronchus that entirely occlude the lumen. The histopathological examination of bronchial biopsy showed evidence of adenoid cystic carcinoma (Figure 3). The patient underwent a left pneumonectomy with node dissection. The surgical margins were negative with no node dissemination. A Chest CT performed seven months following surgery didn’t show tumor recurrence. The patient is regularly seen at our consultation. He reports an improvement of his symptoms after the surgery.

 

Case 2: we report the case of a 52 year-old female with insulin-dependent diabetes who was followed as outpatient during four years for cough, wheezing and dyspnea with progressive worsening. The diagnosis of non-allergic asthma was initially suspected and the patient was treated with a long-acting β2 agonist treatment in association with an inhaled corticosteroid. Despite the regular use of an optimal treatment, her symptoms were worsening. Chest X ray showed a mediastinal widening (Figure 4). Spirometry showed a flattened inspiratory curve (Figure 5). The thoracic CT scan revealed a proximal tracheal solid mass (Figure 6). So a Surgical resection of fours tracheal rings was performed. The histopathological analysis concluded to the diagnosis of adenoid cystic carcinoma with positive surgical margins. The patient received postoperative radiotherapy. She had a postoperative follow up of six months with a clear improvement in dyspnea.

 

 

Discussion Up    Down

Adenoid cystic carcinomas (or cylindroma) are malignant epithelial tumors that have a slow development. It occurs mainly in the salivary glands, but can also affect other sites such as lungs, breasts, skin and uterine cervix [1]. It represents 20 to 35% of tracheal tumors [2]. Primary adenoid cystic carcinoma in the lungs mostly affects the main airway like the trachea or main bronchi. Pulmonary parenchyma involvement is extremely rare [3,4]. It affects both sexes similarly and especially young one aged between 4 th and 5 th decades [1]. Our two patients belong to this interval. There is no evidence of association with tobacco smoking. Clinical manifestations are not specific, dominated mainly by hemoptysis and dyspnea as it was for our patients [5]. Our second case illustrate the example of ACC presenting as asthma. In some cases, the tumor can be asymptomatic resulting in a delayed diagnosis. The histopathological diagnosis is based mainly on the bronchoscopy since the proximal endoluminal growth of the tumor, allowing the realization of a biopsy or a bronchoscope-guided fine-needle aspiration [6]. Hematoxylin-eosin staining remains the principal method used for the diagnosis of ACC.

 

Immunohistochemistry can be useful in some case. Indeed there is three types of growth to be distinguished on histology: cribriform, tubular and solid. There is a correlation between the evolution of ACC and the histological models. The solid pattern is the most aggressive compared to cribriform [7] .For our patients, the first case was a tubular and cribriform ACC whereas it was of solid type for the second case. The treatment is essentially based on surgical resection for early stage. Postoperative radiotherapy is indicated in the case of positive surgical margins and for non-operable patients [8]. Indeed, ACC is characterized by a possible submucosally and perineurally spread. The place of chemotherapy remains a controversial subject. There is some cases described in the literature that had a response to chemotherapy especially in metastatic cases. The prognosis of ACC depends on histological growth patterns, stage and margins of surgical resection. ACC has a good prognosis with a low metastatic risk especially in patients undergoing radical surgical treatment with clear margins [8]. Otherwise, recurrence or metastases may occur [9,10]. Thus, a prolonged period of follow up is needed.

 

 

Conclusion Up    Down

ACC is a tumor of good prognosis provided an early diagnosis and rapid management based essentially on surgical resection associated in some cases with radiotherapy. It has no specific clinical presentation leading often to a late diagnosis. More studies are needed to determine the contribution of chemotherapy especially in metastatic tumors.

 

 

Competing interests Up    Down

The authors declare no competing interests.

 

 

Authors´ contributions Up    Down

All authors participated in the preparation of the manuscript. Naceur Rouatbi and Samah Joobeur conceptualised and designed the manuscript. Ahmed Ben Saad and Rania Kadoussi performed manuscript writing. Manel Njima and Nouha Ben Abdeljelil performed the histopathologic examination. Saousen Cheikh Mhamed and Nesrine Fahem revised the english version. All authors read and approved the final version of the manuscript.

 

 

Figures Up    Down

Figure 1: chest X ray-case 1: persistent left basal consolidation (white star) with a left mediastinal attraction (white arrow)

Figure 2: chest CT scan-case 1: (A) axial CT scan image; (B) coronal CT scan image. Left-sided bronchial-centric mass in the left main bronchus (white arrow) associated with multiple left hilar lymph nodes and carcinomatous lymphangitis in the left inferior lobe

Figure 3: histopathology: (A) adenoid cystic carcinoma showing cribriform and tubular growth of small hyperchromatic epithelial cells (yellow arrow) (HEx40); (B) tumoral cells are cuboidal with scant cytoplasm and homogenous nuclei (yellow arrow) (HEx400)

Figure 4: chest X ray - case 2: mediastinal widening (white arrow)

Figure 5: spirometry: flow volume loop: flattened inspiratory curve (black arrow)

Figure 6: chest CT scan - case 2: (A) axial CT scan image; (B) coronal CT scan image. Proximal tracheal solid mass (yellow arrow)

 

 

References Up    Down

  1. Chhabra S, Bhutani N, Singh S, Batra A, Sen R. Primary adenoid cystic carcinoma of bronchus: Report of a rare case. Human Pathology. 2017;7:53-56. Google Scholar

  2. Fraire AE, Dail DH. Tracheobronchial Tumors of the Salivary Gland Type. Dail and Hammar's Pulmonary Pathology: Volume II: Neoplastic Lung Disease. New York, NY: Springer New York. 2008;398-426. Google Scholar

  3. Mukherjee S, Dattachaudhuri A, Bhanja P, Deb J. A rare case of primary adenoid cystic carcinoma of lung. Indian J Chest Dis Allied Sci. 2014;56(3):175-7. PubMed | Google Scholar

  4. Inoue H, Iwashita A, Kanegae H, Higuchi K, Fujinaga Y, Matsumoto I. Peripheral pulmonary adenoid cystic carcinoma with substantial submucosal extension to the proximal bronchus. Thorax. 1991 Feb;46(2):147-8. PubMed | Google Scholar

  5. zaibi H, ben amar J, Frikha H. Le carcinome adénoide kystique primitif, une tumeur bronchique rare. La tunisie médicale. 2016;94(11):698.

  6. Qiu S, Nampoothiri MM, Zaharopoulos P, Logrono R. Primary pulmonary adenoid cystic carcinoma: report of a case diagnosed by fine-needle aspiration cytology. Diagn Cytopathol. 2004 Jan;30(1):51-6. PubMed | Google Scholar

  7. Ratto GB, Alloisio A, Costa R, Chiaramondia M. Primary peripheral adenoid cystic carcinoma of the lung. A case report. Acta Chir Belg. 2003 Aug;103(4):414-5. PubMed | Google Scholar

  8. Yousem SA, Nicholson AG. Adenoid cystic carcinoma. WD Travis, E Brambilla, HK Müller-Hermelink, CC Harris (Eds) Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. Lyon, France: IARC Press, 2004:64-6.

  9. Hu MM, Hu Y, He JB, Li BL. Primary adenoid cystic carcinoma of the lung: Clinicopathological features, treatment and results. Oncol Lett. 2015 Mar;9(3):1475-81. PubMed | Google Scholar

  10. Kumar N S, Iype EM, Thomas S, Sankar UV. Adenoid Cystic Carcinoma of the Trachea. Indian J Surg Oncol. 2016;7(1):62-66. PubMed | Google Scholar

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 


The Pan African Medical Journal articles are archived on Pubmed Central. Access PAMJ archives on PMC here

Volume 34 (September - December 2019)

Article tools

PDF (826 Kb)
Contact the corresponding author
Download to Citation Manager
EndNote
Reference Manager
Zotero
BibTex
ProCite


Keywords

Adenoid cystic carcinoma
Salivary gland cancer
Lung cancer
Bronchogenic carcinoma

Rate this article

Altmetric

PAMJ is a member of the Committee on Publication Ethics
PAMJ Authors services
Next abstract

PAMJ is published in collaboration with the African Field Epidemiology Network (AFENET)
Currently tracked by: DOAJ, AIM, Google Scholar, AJOL, EBSCO, Scopus, Embase, IC, HINARI, Global Health, PubMed Central, PubMed/Medline, Ulrichsweb, More to come . Member of COPE.

ISSN: 1937-8688. © 2019 - Pan African Medical Journal. All rights reserved