MALT Lymphoma of Minor Salivary Glands in a Sjögren's Syndrome Patient: a Case Report and Review of Literature

Abstract

Background: Sjögren's syndrome is a chronic systemic disease, characterized by lymphocytic infiltration and destruction mainly of the salivary and lacrimal glands, resulting in xerostomia and xeropthalmia. Sjögren's syndrome patients have a 44-fold excess risk for the development of non-Hodgkin's lymphoma particularly mucosa-associated lymphoid tissue (MALT) lymphoma, prevalently affecting the major salivary glands. In this report, a rare case of MALT lymphoma of minor salivary glands in a patient with Sjögren's syndrome is described. A review of the published cases of MALT lymphoma located in the minor salivary glands of patients with Sjögren's syndrome is provided.

Methods: In a 64-year-old female patient previously diagnosed with Sjögren's syndrome, an asymptomatic soft tissue mass at the palate was noticed, exhibiting rapid enlargement within one month. With a main differential diagnosis of salivary gland neoplasm or lymphoproliferative lesion, a partial biopsy was performed accompanied by proper immunohistochemical analysis.

Results: A final diagnosis of MALT lymphoma was rendered and the patient was referred for further multidisciplinary evaluation. Gastric endoscopy and biopsy revealed a Helicobacter pylori-negative gastric MALT lymphoma, while spleen involvement and bone marrow infiltration were also identified. Patient was classified as having stage IV disseminated disease and a standard chemotherapy protocol was administered; the treatment was well tolerated and resulted in complete remission.

Conclusions: This case emphasizes the need for close monitoring of patients with Sjögren's syndrome by oral medicine specialists, which, besides ensuring proper management of xerostomia and its sequelae, may also lead to early recognition of lymphoma development.

Keywords: MALT lymphoma; Sjögren’s syndrome; minor salivary glands.

Figure 1

Asymptomatic mass noticed at the junction of hard and soft palate (arrow).

Figure 2

Dense, diffuse infiltration of a minor salivary gland by a monotonous population of lymphoid cells, destroying the normal architecture. An organized lymphoid follicle (arrows) and lymhoepithelial islands (yellow arrow) are also observed (haematoxylin and eosin stain, original magnification x100).

Figure 3

Infiltration by numerous centrocyte-like cells (yellow arrows) is observed; scattered blast cells are also noted (arrows) (haematoxylin and eosin stain, original magnification x400).

Figure 4

Neoplastic cells exhibiting positivity for anti-CD20 (original magnification x100).

Figure 5

Strong positivity for anti-BCL2 (original magnification x100).