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An extremely rare tracheal tumor in coronavirus disease-2019 screening: Marginal zone lymphoma

1 Department of General Thoracic Surgery and Lung Transplantation, Ankara City Hospital, University of Health Sciences, Ankara, Turkey
2 Department of Pathology, Ankara City Hospital, University of Health Sciences, Ankara, Turkey

Date of Submission17-Sep-2021
Date of Acceptance11-Oct-2021
Date of Web Publication14-Jan-2022

Correspondence Address:
Muhammet Ali Beyoglu,
B1 floor, Cardiovasculary Surgery building, City Hospital, 1604. street, Number 9, Cankaya, Ankara
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_1645_21

 > Abstract 

Mucosal-associated lymphoid tissue lymphoma is extremely rare due to the scarcity of lymphoid tissue in the trachea. To date, approximately 20 cases of tracheal mucosa-associated lymphoid tissue lymphoma have been reported. This case report presents a primary tracheal extranodal marginal zone lymphoma case detected incidentally during the coronavirus disease-2019 screening.

Keywords: Coronavirus disease-2019, lymphoma, marginal zone, mucosa-associated lymphatic tissue

How to cite this URL:
Beyoglu MA, Sahin FM, Albayrak A, Yekeler E. An extremely rare tracheal tumor in coronavirus disease-2019 screening: Marginal zone lymphoma. J Can Res Ther [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=335486

 > Introduction Top

Marginal zone (MZ) lymphomas arise from B-lymphocytes in the MZ located in the external part of secondary lymphoid follicles. They are low-grade non-Hodgkin lymphomas with a painless clinical course.[1] The World Health Organization divides MZ lymphomas into splenic, nodal, and extranodal MZ lymphomas (EMZL).[1] EMZL, also known as mucosa-associated lymphatic tissue lymphoma, is often diagnosed in the stomach and gastrointestinal tract but can occur in various extragastric regions. EMZL outside the gastrointestinal tract is most commonly seen in the lungs.[2] Primary tracheal lymphoma is rarely seen due to the scarcity of lymphoid tissue in the trachea. To date, approximately 20 cases with tracheal EMZL have been reported in the literature. In the present case report, a case of tracheal MZ lymphoma incidentally detected in the thorax computed tomography (CT) of a patient who applied with positive complaints during the coronavirus disease-2019 (COVID-19) pandemic is presented.

 > Case Report Top

A 53-year-old male patient applied to the emergency department with complaints of chills, fever, and cough. In the thorax CT taken with the suspicion of COVID-19, irregular and asymmetrical wall thickening was detected in the anterolateral wall of the trachea [Figure 1]a. He was referred to our hospital for the further management. The patient had no history of fever, night sweats, or weight loss in the last 6 months. In the fiberoptic bronchoscopy performed on the patient, a 15 mm × 5 mm mass protruding into the lumen was observed approximately 2 cm below the cricoid cartilage. The mucosal surface had a regular appearance [Figure 2]a and [Figure 2]b. Multiple biopsies were taken from the lesion. After bronchoscopy, the patient underwent positron emission tomography for whole-body scanning. No hypermetabolic area was observed in the lesion, which persisted as millimetric wall thickening after the biopsy procedure and in other areas of the body [Figure 1]b. Histopathological examination revealed dense infiltrations of monomorphic lymphoid tumor cells in the lamina propria of the bronchial mucosa and lymphoepithelial lesions in the submucosal gland duct [Figure 3]a. CD20 (+), BCL2 (+), CD5 (−), CD10 (−), CD23 (−), and cyclin D1 (−) were detected in the immunohistochemical study [Figure 3]b. The patient was diagnosed with primary EMZL of the trachea. Surgical treatment, radiotherapy, or rituximab treatments were recommended to the patient due to the locality of the disease and the lack of sufficient literature to evaluate the most appropriate treatment. After the decision of the patient, he was referred to medical oncology for rituximab treatment.
Figure 1: (a) In the initial thorax computed tomography (CT), asymmetrical thickening of the tracheal wall is noted (Tracheal tumor marked with white arrow), (b) no increased metabolic involvement is observed in the lesion in positron emission tomography-CT taken for whole-body scanning after bronchoscopic biopsy (Tracheal tumor area after biopsy is marked with white arrow)

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Figure 2: (a) Bronchoscopic appearance of the tumor placed in the proximal trachea, (b) a lobulated mass is observed under the regular mucosa

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Figure 3: (a) Intense lymphocytic infiltration is seen under the bronchial mucosa in hematoxylin and eosin-stained slides (H and E, ×100), (b) CD20 immunostaining highlighting the neoplastic B cells (anti-CD20, ×40)

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 > Discussion Top

While mucosa-associated lymphoid tissue lymphoma is a common type of primary pulmonary carcinoma, it is scarce in the trachea. Patients with tracheal EMZL usually present with shortness of breath, wheezing, stridor, or coughing. Since EMZLs are painless lesions, they are detected incidentally either when they cause symptoms in the late period or during routine controls. In the present case report, the patient complained of fever and cough during the COVID-19 pandemic, and a tracheal lesion was detected incidentally in the evaluation.

Narrowing in the air column on direct X-ray can only be noticed in advanced tracheal lesions. In patients presenting with ambiguous complaints, thoracic CT should be performed if there is a suspicion of an obstructing mass in the trachea after clinical evaluation. Thorax CT findings may include asymmetric tracheal wall thickening, circumferential tracheal lesion, or single or multiple polypoid lesion.

The gold standard diagnostic option in tracheal EMZL is bronchoscopy. During bronchoscopy, the appearance of a smooth, broad-based mass lesion that narrows the lumen is typical for EMZL. Generally, a single fleshy polypoid lesion is seen, although multiple tracheal lesions have also been reported (Kang, Minami).[3],[4] Multiple mucosal biopsies should be taken for the diagnosis. An immunohistochemical study with a sufficient amount of bronchoscopic biopsy material is diagnostic for EMZL.

The literature on the treatment of tracheal EMZLs usually consists of case reports. While there are reports of successful results of classical treatments such as surgical treatment, bronchoscopic local excision, radiotherapy and chemotherapy, there are also reports reporting successful results of using immunotherapy (rituximab) alone or in combination with chemotherapy.[5],[6],[7],[8],[9]

In conclusion, primary tracheal EMZL is rare but has a favorable prognosis. The available data in the literature are not sufficient to compare different treatment options. Various treatment options such as surgical resection, radiotherapy, bronchoscopic excision, chemotherapy, immunotherapy (rituximab), and immunochemotherapy are effective in treating this disease. In the ongoing COVID-19 pandemic, a large number of patients are undergoing thoracic imaging. EMZL should also be considered in the differential diagnosis of isolated tracheal lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC; 2008.  Back to cited text no. 1
Zucca E, Bertoni F. The spectrum of MALT lymphoma at different sites: Biological and therapeutic relevance. Blood 2016;127:2082-92.  Back to cited text no. 2
Kang JY, Park HJ, Lee KY, Lee SY, Kim SJ, Park SH, et al. Extranodal marginal zone lymphoma occurring along the trachea and central airway. Yonsei Med J 2008;49:860-3.  Back to cited text no. 3
Minami D, Ando C, Sato K, Moriwaki K, Sugahara F, Nakasuka T, et al. Multiple mucosa-associated lymphoid tissue lymphoma of the trachea. Intern Med 2017;56:2907-11.  Back to cited text no. 4
Okubo K, Miyamoto N, Komaki C. Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea: A case of surgical resection and long term survival. Thorax 2005;60:82-3.  Back to cited text no. 5
Raimundo S, Alexandre A, Pinto C. Tracheal Secondary Involvement by mucosa-associated lymphoid tissue Lymphoma - A Rare Diagnosis. Arch Bronconeumol 2016;52:567-8.  Back to cited text no. 6
Mira-Avendano I, Cumbo-Nacheli G, Parambil J. Mucosa-associated lymphoid tissue lymphoma of the trachea. J Bronchology Interv Pulmonol 2012;19:44-6.  Back to cited text no. 7
Li Y, Jiang J, Herth FJ, Wan T, Zhang R, Xiao M, et al. Primary tracheal mucosa – Associated lymphoid tissue lymphoma treated with a water-jet hybrid knife: A case report. Respiration 2019;97:168-72.  Back to cited text no. 8
Mizuno S, Ota S, Tanaka T, Shiomi K, Matsumura T, Kishimoto N. Primary tracheal malignant lymphoma detected during a regular checkup in an asbestos dust-exposed smoker. Acta Med Okayama 2014;68:177-81.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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