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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 364-367
Primary non-Hodgkin's lymphoma of the salivary gland: A spectrum of lymphoepithelial sialadenitis, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue with transformation to high-grade lymphoma


Department of Pathology, Grant Medical College, Mumbai, India

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Date of Web Publication12-Jun-2010
 

   Abstract 

Lymphoid infiltrates of the salivary gland can be either reactive or neoplastic. The reactive lesion, lymphoepithelial sialadenitis (LESA) may be associated with Sjogren's syndrome (SS) or may occur as an isolated salivary gland enlargement. Patients with LESA/SS have a particularly high risk of subsequently developing lymphoma, which is a low-grade mucosa-associated lymphoid tissue (MALT) type lymphoma of the salivary gland. We document a rare case of primary non-Hodgkin's lymphoma of the parotid gland arising in the background of LESA and with a rare example of transformation from low grade to high-grade B cell lymphoma of MALT type.

Keywords: High-grade non-Hodgkin′s lymphoma, low-grade MALT lymphoma, primary lymphoma, salivary gland

How to cite this article:
Agale SV, D'Costa GF, Hastak MS, Shedge RT. Primary non-Hodgkin's lymphoma of the salivary gland: A spectrum of lymphoepithelial sialadenitis, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue with transformation to high-grade lymphoma. Indian J Pathol Microbiol 2010;53:364-7

How to cite this URL:
Agale SV, D'Costa GF, Hastak MS, Shedge RT. Primary non-Hodgkin's lymphoma of the salivary gland: A spectrum of lymphoepithelial sialadenitis, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue with transformation to high-grade lymphoma. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Nov 14];53:364-7. Available from: http://www.ijpmonline.org/text.asp?2010/53/2/364/64351



   Introduction Top


Lymphoid proliferation of the salivary gland includes a spectrum of disorders ranging from benign myoepithelial sialadenitis (MESA) also known as lymphoepithelial sialadenitis (LESA) to overt low and high-grade B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) type. [1],[2],[3],[4],[5],[6],[7],[8],[9]

Primary lymphomas arising in the salivary glands are uncommon with an incidence of 1.5 to 5% of all salivary gland neoplasms. [6],[7],[9] The majority (70-80%) involve the parotid gland [2],[4],[8] and are low-grade lymphomas. [1],[2],[4],[6] This low-grade lymphoma usually arises in a background of LESA. [3],[5],[6]

LESA/MESA is a morphological feature of Sjogren's syndrome but may also be found in patients without the clinical picture of Sjogren's syndrome and lead to development of lymphoma. [1],[5]


   Case Report Top


A 45-year-old female presented with right-sided parotid swelling since five months. The patient received antituberculous treatment based on FNAC diagnosis but did not respond to it. Subsequent biopsy was reported as pleomorphic adenoma and the patient underwent partial parotidectomy. On examination the parotid swelling was firm, painless, measuring 7.5 x 4.5 x 3 cm. There was no clinical evidence of Sjogren's syndrome or lymphadenopathy. USG of abdomen and pelvis was normal.

Gross Examination

The partial parotidectomy specimen measured 7.5 x 4.5 x 3 cm. External surface was nodular. The cut surface revealed solid, grayish white, fleshy, soft to firm tumor involving the salivary gland tissue with an area of necrosis [Figure 1]a.

Microscopic Examination

The histomorphology revealed a spectrum of LES to low-grade lymphoma with transformation to high-grade lymphoma. The low-grade B-cell MALT-type lymphoma showed a dense lymphoid infiltrate with obliteration of acini and formation of a localized mass. The lymphoepithelial lesions were prominent with the presence of monocytoid B-cells {centrocyte-like (CC-L) cells} which formed broad halos around the epithelial nests and extended away from them in broad strands thus linking together several lymphoepithelial lesions [Figure 2].

The high-grade component was composed of large cells (three times the small lymphocytes) with moderate amount of eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli and nuclear lobulations. Binucleate cells were present. The cells had moderately high mitotic rate [Figure 3].

The LES was localized to the peripheral part of the tumor characterized by lymphoid infiltrate with follicular hyperplasia, surrounding and infiltrating the salivary ducts, with disorganization and proliferation of ductal cells to form lymphoepithelial lesions [Figure 1]a.

Immunohistochemistry

The lymphoid cells were positive for leucocyte common antigen (LCA) and CD 20 (B-cell marker) and negative for cytokeratin and epithelial membrane antigen (EMA).


   Discussion Top


Primary malignant lymphomas of the salivary gland constitute about 4-5% of all extranodal lymphomas. [2],[7],[9] The majority of the salivary gland lymphomas (70-80%) arise in the parotid gland and most are low-grade non-Hodgkin lymphomas. [2],[7],[10] According to Batsakis [10] this propensity towards the parotid gland may be due to its embryogenesis, with lymph nodes and lymphoid tissue being commonly found within the gland proper.

Primary parotid lymphomas represent 1-4% of all parotid tumors [2],[10] and approximately 20% are associated with SS or LESA/MESA [2],[5] which is an autoimmune disease.

Many studies over the years have shown an association between LESA/SS and lymphoma. [2],[3],[4],[5],[6] Patients with LESA/SS are typically female (80%) with an age range of 24 to 88 years [3] and have a 44-fold increased risk of developing salivary gland or extra-salivary lymphomas. [5],[9] Notably, most of them are marginal zone B-cell lymphomas of the MALT type. [5],[8]

Women have lymphomas of the salivary glands more frequently than men, especially if one considers those lymphomas that arise in association with SS or LESA with a peak age incidence in the sixth and seventh decades of life. [1],[3] Our case was also a female of 45 years and presented with a parotid mass.

Hymann and Wolff [1] have suggested the following criteria for a lesion to be considered a primary parotid lymphoma: 1) Involvement of the gland should be the first manifestation of the disease. 2) Histologically, the disease should involve the gland parenchyma and not the adjacent lymph nodes. 3) There should be confirmation of the malignant nature of lymphoid infiltrate. All the three criteria were fulfilled in our case.

Both LESA and MALT lymphoma are known to present as salivary gland enlargement. In LESA the patient presents with recurring and persistent, firm swelling of the affected gland which may be associated with discomfort or pain. [5],[6] Lymphoma produces a firm to hard swelling of the affected gland which has been noted less than six months in 80% of the cases .[2] In this case the patient presented with persistent, painless, firm swelling which was of five months duration.

On gross examination lymphomas usually are well-circumscribed lesions with grayish white to pink-tan cut surface and soft to firm, rubbery consistency. [1] The parotidectomy specimen we received had more or less similar gross morphology.

The spectrum of histopathologic features of LESA/MESA includes two different patterns for which the terms "fully benign lymphoid infiltrate" and "lymphoproliferative lesions" have been proposed. [4] In the "fully benign lymphoid infiltrate" the lobular architecture of the gland is preserved, lymphoepithelial lesions are prominent and monocytoid and/or marginal zone B-cells (CC-L cells) are restricted to the lymphoepithelial lesions. It also displaces prominent lymphoid follicles without expansion of the marginal or mantle zone with presence of small lymphocytes and plasma cells in the interfollicular regions. [4],[5]

In the second histomorphologic pattern of LESA/MESA, termed "lymphoproliferative lesion" the process is diffuse or multifocal within the gland islands of normal acini are often preserved and aggregates of CC-L cells may be present within the diffuse lymphoid infiltrate. [4],[5] MALT lymphoma of the salivary gland typically produces a dense lymphoid infiltrate with obliteration of acini, diffusely involves the gland or may form a localized mass. Both in MALT-type lymphoma of the salivary gland and in LESA/MESA, reactive lymphoid follicles and lymphoepithelial lesions are prominent, whereas only in MALT-type lymphoma but not in LESA/MESA, CC-L cells formed broad halos around epithelial nests and broad strands between "lymphoepithelial lesions". [3],[4] The characteristic histomorphologic features of LESA and low-grade MALT lymphoma with high-grade component was present in our case.

The most important differential diagnosis of LESA is MALT lymphoma and vice versa. The important features in the differential diagnosis with MALT lymphoma are the number and distribution of monocytoid B-cells (as described above), the presence of sheets of plasma cells with or without Dutcher bodies and soft tissue and perineural invasion. [3],[4],[5],[6]

The high-grade B-cell lymphoma of MALT type has to be differentiated from other poorly differentiated malignant tumors, particularly lymphoepithelioma like carcinoma and melanoma. Demonstrations of leucocyte-associated antigens (CD 45, CD 20) and absence of cytokeratins and other nonlymphoid markers can be useful in the differential diagnosis. [5] The cell positivity for LCA and CD 20 (B-cell marker) and negativity for cytokeratin confirmed the diagnosis of lymphoma of B-cell origin, in this case. According to Carbone et al.,[4] there is a pathological and clinical spectrum of the salivary gland lymphoid proliferations, that is, from benign-appearing, polyclonal to monoclonal lesions to low-grade lymphomas to aggressive high-grade B-cell lymphomas. This concept is observed and reinforced in the present case.

It is always sensible to make a distinction between malignant lymphomas that appear in the course of LESA with or without Sjogren's syndrome and lymphomas that developed in salivary gland of patients without immune disease, for the reason that approximately 10-45% of patients with LESA or SS with salivary gland lymphoma eventually develop overt extra-salivary lymphoma. [5]

Salivary gland lymphomas have a better survival rate as compared to nodal and other extra-nodal lymphomas. Our patient had a low-grade B-cell lymphoma of MALT type with transformation to high-grade B-cell lymphoma and the tumor was localized to the salivary gland. The patient was alright six months after the treatment. It is imperative to do a follow-up in such patients as 10-45% eventually develop overt extra-salivary lymphoma. [5] Lymphoma rarely presents as a mass in the parotid region and must be considered in the differential diagnosis of discrete or diffuse swellings of the parotid gland whether it occurs unilaterally or bilaterally. In order to establish a suspected diagnosis of salivary gland lymphoma one should examine not only a biopsy but the entire specimen of the salivary gland and preferably adjacent lymph nodes, as well.

 
   References Top

1.Hyman GA, Wolff M. Malignant lymphomas involving the salivary glands. Am J Clin Pathol 1976;65:412-38.  Back to cited text no. 1      
2.Gleeson MJ, Bennett MH, Czwson RA. Lymphomas of salivary glands. Cancer 1986;58:699-704.  Back to cited text no. 2      
3.Quintana PG, Kapadia SB, Bahaler DW, Johnson JT, Swerdlow H. Salivary gland lymphoid infiltrates associated with lymphoepithelial lesions: A clinicopathologic, immunophenotypic and genotypic study. Hum Pathol 1997;28:850-61.  Back to cited text no. 3      
4.Carbone A, Gloghini A, Fertito A. Pathological features of lymphoid proliferations of the salivary glands: Lymphoepithelial sialadenitis versus low grade B-cell lymphoma of MALT type. Ann Otol Rhinol Laryngol 2000;109:1170-5.  Back to cited text no. 4      
5.Harris NL. Lymphoid proliferations of the salivary glands. Am J Clin Pathol 1999;111:S94-103.  Back to cited text no. 5  [PUBMED]    
6.Hyjek E, Smith W, Isaacson P. Primary B cell lymphoma of salivary gland and its relationship to myoepithelial sialadenitis (MESA). Hum Pathol 1988;19:766-76.  Back to cited text no. 6      
7.Mehle ME, Krans DH, Wood BG, Tubbs R, Tucker HM, Lavertu P. Lymphoma of the parotid gland. Laynogoscope 1993;103:17-21.  Back to cited text no. 7      
8.Dunn P, Kuo TT, Shin LY, Lin TC, Wang PN, Kuo MC, et al. Primary salivary gland lymphoma: A clinicopathologic study of 23 cases in Taiwan. Acta Haematol 2004;112:203-8.  Back to cited text no. 8      
9.Roh JL, Huh J, Suh C. Primary non-Hodgkin lymphomas of the major salivary glands. J Surg Oncol 2008;97:35-9.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Batsakis JG. Primary lymphomas of the salivary glands. Ann Otol Rhinol Laryngol 1986;95:107-8.  Back to cited text no. 10  [PUBMED]    

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Correspondence Address:
Shubhangi Vinayak Agale
Department of Pathology, Grant Medical College, Mumbai-400 008,
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.64351

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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