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CASE REPORT
Year : 2018  |  Volume : 21  |  Issue : 3  |  Page : 392-394

Pulmonary extranodal marginal zone B-cell lymphoma of mucosa-associated lymph tissue: A case report and literature review


Department of Thoracic Surgery, School of Medicine, Second Affliated Hospital, Zhejiang University, Hangzhou, Zhejiang Province, China

Date of Acceptance04-Sep-2017
Date of Web Publication09-Mar-2018

Correspondence Address:
Dr. Y Chai
Department of Thoracic Surgery, School of Medicine, Second Affiliated Hospital, Zhejiang University, 88 Jiefang Road, Hangzhou 310009
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_393_16

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   Abstract 


Extranodal marginal zone B-cell lymphoma of mucosa-associated lymph tissue (MALT lymphoma) is a rare subtype of marginal zone B-cell lymphoma. Most primary MALT lymphoma occurs in the stomach, while lung is an uncommon site of MALT lymphoma. We herein report a case of MALT lymphoma in a 44-year-old female who underwent a pulmonary lobectomy and received rituximab plus CHOP chemotherapy regimen. The patient had an uneventful postoperative course and a good prognosis without tumor recurrence for 3 years.

Keywords: Extranodal marginal zone B-cell lymphoma, mucosa-associated lymphoid tissue lymphoma, pulmonary lymphoma


How to cite this article:
Wu Y, Zhao L, Chai Y. Pulmonary extranodal marginal zone B-cell lymphoma of mucosa-associated lymph tissue: A case report and literature review. Niger J Clin Pract 2018;21:392-4

How to cite this URL:
Wu Y, Zhao L, Chai Y. Pulmonary extranodal marginal zone B-cell lymphoma of mucosa-associated lymph tissue: A case report and literature review. Niger J Clin Pract [serial online] 2018 [cited 2019 Nov 14];21:392-4. Available from: http://www.njcponline.com/text.asp?2018/21/3/392/226969




   Introduction Top


Mucosa-associated lymph tissue (MALT) lymphoma is a form of mature lymphoma originating from B-cells in the marginal zone of the MALT, and it constitutes 9% of the mature B-cell lymphoma. The stomach is the most common primary site of MALT lymphoma, followed by conjunctiva and orbit.[1] A patient with infrequent pulmonary MALT lymphoma was found in our institution. The patient underwent pulmonary lobectomy and received rituximab plus CHOP (R-CHOP) chemotherapy regimen after surgery. The patient had an uneventful postoperative course and a good prognosis without tumor recurrence for 3 years.


   Case Report Top


A 44-year-old female was admitted to our hospital for cough and expectoration for 2 months. No significant abnormalities were identified on physical examination. The patient had no past medical history, and physical examination did not yield any remarkable findings. Serum levels of tumor markers were within normal limits such as carcinoembryonic antigen, CA21-1, neuron-specific enolase, and squamous cell carcinoma. The computed tomography (CT) scan identified a mixed ground-glass patchy shadow (2 cm) on the right superior lobar [Figure 1]. No enlarged lymph nodes or distant metastasis were detected on radiography. After discussion with radiologists, we tend to diagnose the patient with pulmonary infection at the beginning, so we asked the patient to receive anti-infective therapy first. However, it did not work, and the occupied disease did not shrink at all. The patient refused CT-guided needle biopsy and asked for surgical treatment. Accordingly, we could not infer to a definite diagnosis before surgery. Finally, the patient received an intraoperative biopsy, and the fast frozen pathology confirmed lymphoma, and then, the tumor was completely resected through pulmonary lobectomy.
Figure 1: The computed tomography scan identified a mixed ground-glass patchy shadow (2 cm) on the right superior lobar

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Two masses were detected in the right superior lobe. Grossly, the two masses measured 1.1 cm × 0.6 cm and 1.5 cm × 1.2 cm in size, respectively. Histologically, the tumor consisted of diffuse proliferative small lymphocytic lymphoma [Figure 2]. Immunohistochemically, CD3, CD5, CD13, CD43, MUM-1, and Bcl-6 were scattered positive in lymphoid cells and CD20 was diffuse and strong positive [Figure 3], whereas staining yielded negative result for CD10 and Cyclin D. The positive expression rate of Ki-67 was 2%. Based on these features, the diagnosis of MALT lymphoma was made.
Figure 2: The tumor consisted of diffuse proliferative small lymphocytic lymphoma

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Figure 3: CD20 was diffuse and strong positive in lymphoid cells

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The patient had a smooth postoperative course. One month after surgery, the patient received three courses of R-CHOP chemotherapy regimen and had a good prognosis without tumor recurrence for 3 years.


   Discussion Top


MALT lymphoma is a form of lymphoma involving the mucosa-associated lymphoid tissue. The primary lymphoma can involve the mucosa-associated lymphoid tissue, frequently of the stomachs, but virtually, any mucosal site can be afflicted. The most common primary site of MALT lymphoma is stomach (45%), followed by conjunctiva (14%) and orbit (11%).[1] Pulmonary MALT lymphoma is quite uncommon, and it was first reported by Herbert in 1983.[2]

Most MALT lymphomas are considered to be related to autoantigens and microbial pathogens.[3] Many kinds of chronic infections seem responsible for MALT lymphoma such as Helicobacter pylori infection for stomach and Epstein-Barr virus infection. However, there is no evidence that pulmonary MALT lymphoma has relationship with autoantigens and microbial pathogens.[4]

On the CT scan, the most common presentation of MALT lymphoma includes findings such as masses with well-defined margins or with air bronchogram.[5] In our case, the CT scan just showed a nonspecific patchy shadow on the right superior lobar without any characteristic imaging features. Therefore, we could not exclude lung cancer and chronic infections by imaging examination before surgery. Some literature have reported that 18F-fluorodeoxyglucose-positron emission tomography-CT may help us to entertain a diagnosis of MALT lymphoma,[6],[7] but further prospective researches are needed to prove its effectiveness in the diagnosis of pulmonary MALT lymphoma.

Histologically, MALT lymphoma is composed of heterogeneous small B-cells. MALT lymphoma does not have a specific immunohistochemistry pattern to make a definite diagnosis at present.[8] The most common immunophenotypes are CD20+, CD79a+, CD5−/+, CD10−, CD23−, CD43+/−, BCL6−, and MUM1−/+.[1] However, immunophenotypes of diffuse large B-cell lymphoma transformed from MALT lymphoma have not been reported previously. In our case, CD20, CD3, and CD5 were positive in lymphoid cells but negative in CD10, indicating the diagnosis of T-cell-rich small B-cell lymphoma. After excluding other B-cell lymphoma by morphology, we infer to the diagnosis of MALT lymphoma.

Pulmonary MALT lymphoma usually has a good prognosis. The 5-year overall survival (OS) of MALT lymphoma can exceed 90%.[4] Therapeutic strategies include surgical resection followed by chemotherapy, surgery resection along, and chemotherapy along. Clarithromycin has also been reported to treat MALT lymphoma. However, there is a controversy about the necessity of surgery in the treatment of MALT lymphoma. Indeed, there is no consensus of the optimum treatment at present. It is reported that patients who had received surgical resection do not have a better 5-year OS.[9] Whereas most patients with pulmonary MALT lymphoma who had received surgical resection had a relatively longer survival, regardless of whether they had received chemotherapy. Kido et al. suggest that it is considerable to perform a radical resection for localized foci.[8] Recently, Vanden Eynden et al. showed that a complete resection is associated with an excellent long-term survival rate.[10] In our case, preoperative examination did not find any metastasis in the patient, giving us the faith to perform a surgery. The patient received a radical surgery resection and had a smooth postoperative course. Nevertheless, this efficacy of this procedure needs further study and follow-up visit.

In conclusion, pulmonary is an uncommon site of MALT lymphoma, and it is really hard to draw a distinction between pulmonary MALT lymphoma and adenocarcinoma before surgery. From our clinical experience, surgical excision is an optimal approach for localized disease because surgery is a safe and technically feasible procedure for both pulmonary MALT lymphoma and adenocarcinoma. It is our hope that our experience may assist future clinicians in their treatment of pulmonary MALT lymphoma.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Maeshima AM, Taniguchi H, Toyoda K, Yamauchi N, Makita S, Fukuhara S, et al. Clinicopathological features of histological transformation from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue to diffuse large B-cell lymphoma: An analysis of 467 patients. Br J Haematol 2016;174:923-31.  Back to cited text no. 1
[PUBMED]    
2.
Herbert A, Wright DH, Isaacson PG, Smith JL. Primary malignant lymphoma of the lung: Histopathologic and immunologic evaluation of nine cases. Hum Pathol 1984;15:415-22.  Back to cited text no. 2
    
3.
Suarez F, Lortholary O, Hermine O, Lecuit M. Infection-associated lymphomas derived from marginal zone B cells: A model of antigen-driven lymphoproliferation. Blood 2006;107:3034-44.  Back to cited text no. 3
    
4.
Borie R, Wislez M, Thabut G, Antoine M, Rabbat A, Couderc LJ, et al. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J 2009;34:1408-16.  Back to cited text no. 4
    
5.
Yoon RG, Kim MY, Song JW, Chae EJ, Choi CM, Jang S, et al. Primary endobronchial marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: CT findings in 7 patients. Korean J Radiol 2013;14:366-74.  Back to cited text no. 5
    
6.
Albano D, Giubbini R, Bertagna F. 18F-FDG PET/CT and primary hepatic MALT: A case series. Abdom Radiol (NY) 2016;41:1956-9.  Back to cited text no. 6
    
7.
Sallak A, Besson FL, Pomoni A, Christinat A, Adler M, Aegerter JP, et al. Conjunctival MALT lymphoma: Utility of FDG PET/CT for diagnosis, staging, and evaluation of treatment response. Clin Nucl Med 2014;39:295-7.  Back to cited text no. 7
    
8.
Kido T, Yatera K, Noguchi S, Sakurai Y, Nagata S, Kozaki M, et al. Detection of MALT1 gene rearrangements in BAL fluid cells for the diagnosis of pulmonary mucosa-associated lymphoid tissue lymphoma. Chest 2012;141:176-82.  Back to cited text no. 8
    
9.
Oh SY, Kim WS, Kim JS, Kim SJ, Kwon HC, Lee DH, et al. Pulmonary marginal zone B-cell lymphoma of MALT type – What is a prognostic factor and which is the optimal treatment, operation, or chemotherapy? Consortium for improving survival of lymphoma (CISL) study. Ann Hematol 2010;89:563-8.  Back to cited text no. 9
    
10.
Vanden Eynden F, Fadel E, de Perrot M, de Montpreville V, Mussot S, Dartevelle P, et al. Role of surgery in the treatment of primary pulmonary B-cell lymphoma. Ann Thorac Surg 2007;83:236-40.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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