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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 2  |  Page : 60-63

Primary non-Hodgkin lymphoma lung: A report of two cases


1 Department of Laboratory Medicine, Aseer Central Hospital, Abha, KSA
2 Department of Pathology, King Khalid University, Abha, KSA

Date of Submission19-Oct-2022
Date of Acceptance16-Nov-2022
Date of Web Publication02-Jan-2023

Correspondence Address:
Dr. Sohaila Fatima
Department of Pathology, King Khalid University, Abha
KSA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ljms.ljms_40_22

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  Abstract 


Lymphomas are a heterogeneous group of malignancies that originate from the neoplastic transformation of lymphocytes. Primary pulmonary non-Hodgkin lymphoma is a rare entity with marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) which is the most common subtype is a low-grade lymphoma accounting for <0.5% of all primary lung neoplasms. The most common presentation is a mass discovered on a chest radiograph in an asymptomatic patient, with symptomatic patients presenting with cough, dyspnea, chest pain, and hemoptysis. On computerized tomography, multiple bilateral lesions are commonly seen in pulmonary MALT lymphoma with consolidation, nodule, and mass being the main morphological patterns. We present two cases diagnosed with marginal zone lymphoma of the lung which is an indolent lymphoma and the most common subtype in the lung.

Keywords: Lung, marginal zone lymphoma, mucosa-associated lymphoid tissue


How to cite this article:
Mohamed Badri RN, Fatima S. Primary non-Hodgkin lymphoma lung: A report of two cases. Libyan J Med Sci 2022;6:60-3

How to cite this URL:
Mohamed Badri RN, Fatima S. Primary non-Hodgkin lymphoma lung: A report of two cases. Libyan J Med Sci [serial online] 2022 [cited 2023 Feb 7];6:60-3. Available from: https://www.ljmsonline.com/text.asp?2022/6/2/60/366080




  Introduction Top


Lymphomas are a diverse group of malignancies that arise from the neoplastic transformation of lymphocytes that have undergone mutations that provide them advantages in terms of proliferation and survival over their normal cellular counterparts. These neoplasms usually originate in lymph nodes or lymphatic tissue in other sites known as extranodal lymphoma and can be localized or widespread at the time of diagnosis.[1] Primary pulmonary non-Hodgkin lymphoma (PPL) is an uncommon kind of lymphoma that accounts for only 0.4% of all lymphomas.[2] The most prevalent histologic types of PPL are marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) and diffuse large B-cell lymphoma.[1] Lymphoma should be considered a differential diagnosis for unresolving lung lesions, and a biopsy should be performed to confirm the diagnosis. Here, we present two cases, one of a 68-year-old female patient diagnosed with difficulty and on histopathology lymphoma and caseating granulomas coexisted and the other of a 71-year-old male patient who was initially diagnosed as bronchiectasis with pneumonia and biopsy proved it to be lymphoma.


  Case Reports Top


Case 1

A 68-year-old female patient presented with a history of shortness of breath for 3 months and right-sided chest pain for a month. Complete blood counts showed hemoglobin – 11.0 g/dL; total leukocyte count – 8.4 × 103/μL with 45% neutrophil, 50% lymphocytes, 01% eosinophil, and 04% monocyte; platelets – 284 × 103/μL, and erythrocyte sedimentation rate – 110 mm in 1 h. Serum chemistry was normal. Screening for viral markers was negative. Sputum for acid-fast bacilli was negative. Computerized tomography (CT) chest revealed an ill-defined heterogeneously enhancing mass lesion of about 9 cm × 6.8 cm × 8 cm, respectively, involving the right upper lobe with trapped air foci; likely due to obstructive bronchiectasis with underlying right upper lobe consolidation collapse. The right upper lobe bronchus was obstructed by the mass. Right mild pleural effusion was noted [Figure 1]. The apical segment of the left lower lobe showed consolidation with an air bronchogram. Left upper lobe posterior segment subpleural (6 mm) nodule was noted. Multiple prominent subcentimetric lymph nodes were seen – mediastinal, paraesophageal, pretracheal, right axillary, subpectoral, and right epiphrenic. Pleural fluid cytology revealed lymphocytic effusion. Bronchoscopic biopsy was repeated twice, but the material was unsatisfactory. Incisional biopsy from the right upper lung mass revealed infiltration of lung tissue by the monotonous population of small-to-medium-sized lymphoid cells with occasional compartmentalization and vague nodularity. Immunohistochemistry revealed tumor cells CD20, BCL2 positivity with CD5, CD 10, CD23, CD 38, BCL6, cyclin D1 negativity consistent with low-grade non-Hodgkin lymphoma (NHL), and extranodal marginal zone lymphoma of MALT [Figure 2]. The nonneoplastic lung tissue showed necrotizing granulomas highly suggestive of tuberculosis. She received steroids for debulking and was planned for rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen.
Figure 1: (a) X-ray showing consolidation of right lobe of lung and nodular lesion in left lobe. (b), (c) CT chest coronal and sagittal sections reveal an ill-defined heterogeneously enhancing mass lesion of 9 cm × 6.8 cm × 8 cm respectively involving the right upper lobe with trapped air foci with underlying right upper lobe consolidation collapse. The right upper lobe bronchus is obstructed by the mass and right mild pleural effusion is seen. CT: Computerized tomography

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Figure 2: (a) Sections showing infiltration of lung tissue by monotonous population of small to medium-sized lymphoid cells with occasional compartmentalization and vague nodularity, (b) Section of lung showing malignant lymphoid cell infiltration along with nonneoplastic lung tissue showing necrotizing granulomas with giant cell (H and E, a and b: ×20). Immunohistochemical study (c) Showing CD20 positivity in malignant cells (CD20, ×20) (d) Showing BCL2 positivity in malignant cells (BCL2, ×20)

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Case 2

A 71-year-old male patient presented with cough and chest pain for 4 months. Complete blood counts and serum chemistry were normal. Screening for viral markers was negative. Sputum for acid-fast bacilli was negative. CT chest revealed bilateral pulmonary areas of consolidation/collapse, fibrotic bands, and bronchiectasis changes, as well as enlarged mediastinal (mainly subcarinal) lymph nodes. Mild pericardial effusion was noted with enlarged splenic hilar lymph nodes. He was given antibiotics but without much improvement and a repeat CT showed no significant changes. Tru-cut biopsy from right-sided lung lesions revealed a monotonous population of small lymphoid cells without any identifiable alveoli. No apoptotic or increase in mitotic activity was seen. Immunohistochemistry revealed tumor cells positive for CD20 and BCL2 and negative for CD5, CD 10, CD23, CD 38, BCL6, and cyclin D1, Ki67 index was low (4%) consistent with low-grade NHL [Figure 3], extranodal marginal zone lymphoma. He was given six cycles of the R-CHOP regimen.
Figure 3: (a) Sections showing infiltration of lung tissue by monotonous population of small-sized lymphoid cells (H and E, ×40). Immunohistochemical study (b). Showing CD20 positivity in malignant cells (CD20, ×10) (c) Showing BCL2 positivity in malignant cells (BCL2, ×20)

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  Discussion Top


PPL is extremely uncommon, accounting for 3%–4% of extranodal NHL, <1% of NHL, and 0.5%–1% of primary pulmonary malignancies.[3] A mass found on a chest radiograph in an asymptomatic patient is the most common presentation, with symptomatic patients presenting with cough, dyspnea, chest discomfort, and hemoptysis.[4] Female predominance was seen in a PPL study, with a mean age of 61.8 years. About 37.5% of patients were asymptomatic at the time of diagnosis, with cough and dyspnea being the most common pulmonary symptoms. A lung infiltration and a large lesion were the most common roentgenographic findings.[2]

Marginal zone lymphomas of the MALT type account for 70%–90% of all cases of PPL, however, they make up <0.5% of all primary lung neoplasms and a low percentage of all lymphomas. The MALT of the bronchus is the source of the majority of primary lymphomas of the lung. Bienenstock and colleagues were the first to report the existence of MALT in the lungs in 1973.[5] MALT is thought to be acquired in response to long-term exposure to antigenic stimuli such as smoking, infection, or autoimmune illness, rather than being a natural component of the human bronchial tree.[6]

MALT-type B-cell lymphomas present as diffuse infiltration of small lymphoid cells and are low-grade. Other small B-cell lymphomas, such as follicular lymphoma, mantle cell lymphoma, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma, are included in the differential diagnosis, especially on small biopsy specimens. At the periphery of masses, lymphoid cells often track along bronchovascular bundles and interlobular septa, whereas alveolar parenchyma is destroyed toward the center. The presence of air bronchograms on high-resolution CT correlates with the presence of intact lungs. It is also possible that central sclerosis is present. Necrosis is quite uncommon.[4] The prevalence of epithelioid granulomas around neoplastic tissues is a well-known occurrence, particularly in lymphomas. Noncaseous epithelioid granulomas are the most common type. An immunological or inflammatory response to tumor-associated antigenic determinants or the generation of cytokines by tumor cells has been attributed to caseous necrosis in granulomas.[7] Our patient had numerous caseating granulomas with giant cells and fibrosis. Individuals with a history of tuberculosis (TB) have a higher risk of NHL, but only if the TB was especially severe and diagnosed a long time ago.[8] There was no such history in our patient. Lymphomas reduce cell-mediated immunity because they predominantly affect the lymphoreticular system.[9] NHL has been discovered to be preceded by chronic inflammatory disease in many cases. However, there is no evidence that Mycobacterium tuberculosis predisposes to NHL.

Multiple bilateral lesions are frequently detected on CT in lung MALT lymphoma. The main morphological patterns are consolidation, nodule, and mass. Other distinguishing characteristics include bronchiectasis (particularly cystic bronchiectasis) and angiogram sign.[10] Both of our cases showed bilateral pulmonary involvement with bronchiectasis, mass, nodules, and consolidatory lesions.

Surgery, systemic treatment, radiation, and antibiotics are all modalities for treating lung marginal zone lymphoma. In cases of localized disease, local therapy (surgery or irradiation) is the treatment of choice. For patients in remission after inadequate surgical excision or with advanced illness, systemic treatment, particularly alkylating-containing regimens, is preferred.[11] In a study, the most common treatment modality was surgical resection in low-stage patients (IE/IIE) and chemotherapy in high-stage patients (IIIE/IVE).[12] One of our patients was given prednisolone for debulking to relieve breathing difficulty. She was planned for chemotherapy with an R-CHOP regimen. Other patients completed six cycles of chemotherapy.

In conclusion, NHL of the lung is rare with marginal zone B-cell lymphomas of the MALT type being the most prevalent histologic subtype observed in this region. Lymphoma must be considered in the differential diagnosis in unresolving lung lesions. Radiology can aid in diagnosis but biopsy is confirmatory. Treatment modalities depend on the stage of the disease.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Press OW, Lichtman MA. General considerations for lymphomas: Epidemiology, etiology, heterogeneity, and primary extranodal disease. In: Kaushansky K, Lichtman MA, Prchal J, Levi MM, Press OW, Burns LJ, et al., editors. Williams Hematology. 9th ed. Newyork, United States: McGraw Hill Education/Medical;2016.  Back to cited text no. 1
    
2.
Ferraro P, Trastek VF, Adlakha H, Deschamps C, Allen MS, Pairolero PC. Primary non-Hodgkin's lymphoma of the lung. Ann Thorac Surg 2000;69:993-7.  Back to cited text no. 2
    
3.
Ferraro P, Trastek VF, Adlakha H, Deschamps C, Allen MS, Pairolero PC. Primary non-Hodgkin's lymphoma of the lung. Ann Thorac Surg 2000;69:993-7.  Back to cited text no. 3
    
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Nicholson AG, Jaffe ES, Guinee D. Lymphohistiocytic tumors. WHO classification of tumours of lung, pleura, thymus and heart. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. World Health Organization Classification of Tumours. 4th ed. Lyon, France: IARC; 2015.  Back to cited text no. 4
    
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Bienenstock J, Johnston N, Perey DY. Bronchial lymphoid tissue. I. Morphologic characteristics. Lab Invest 1973;28:686-92.  Back to cited text no. 5
    
6.
Nicholson AG, Wotherspoon AC, Diss TC, Hansell DM, Du Bois R, Sheppard MN, et al. Reactive pulmonary lymphoid disorders. Histopathology 1995;26:405-12.  Back to cited text no. 6
    
7.
Hall PA, Kingston J, Stansfeld AG. Extensive necrosis in malignant lymphoma with granulomatous reaction mimicking tuberculosis. Histopathology 1988;13:339-46.  Back to cited text no. 7
    
8.
Amlot PL. Cell mediated Immunity in Lymphomas. In: Jones DB, Wright DH, editors. Lymphoproliferative Diseases; Immunology and Medicine Series. Vol. 15. Springer, Dordrecht. 1990. p. 73-90. https://doi.org/10.1007/978-94-009-0739-3_6.  Back to cited text no. 8
    
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Askling J, Ekbom A. Risk of Non-Hodgkin's lymphoma following tuberculosis. Br J Cancer 2001;84:113-5.  Back to cited text no. 9
    
10.
Deng W, Wan Y, Yu JQ. Pulmonary MALT lymphoma has variable features on CT. Sci Rep 2019;9:8657.  Back to cited text no. 10
    
11.
Sammassimo S, Pruneri G, Andreola G, Montoro J, Steffanoni S, Nowakowski GS, et al. A retrospective international study on primary extranodal marginal zone lymphoma of the lung (BALT lymphoma) on behalf of International Extranodal Lymphoma Study Group (IELSG). Hematol Oncol 2016;34:177-83.  Back to cited text no. 11
    
12.
Lee H, Yang B, Nam B, Jeong BH, Shin S, Zo J, et al. Treatment outcomes in patients with extranodal marginal zone B-cell lymphoma of the lung, J Thorac Cardiovasc Surg, 2017;154:pp. 342-9. https://doi.org/10.1016/j.jtcvs.2017.03.043.  Back to cited text no. 12
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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