A teenager followed since the age of 14 years for intermittent swelling of the knee with negative image and laboratory investigations. She developed SS at the age of 18 year showing intermittent swelling of parotid glands, positive RF, antinuclear antibodies (ANA) and anti-SSA antibodies, elevated erythrocyte sedimentation rate (ESR, 25mm/1h), but normal serum level of cryoglobulins, IgG4 and complement. The Schirmer test and serologic testing for virus were negative. The minor SG biopsy showed focal lymphocytic sialadenitis but the histopathologic diagnosis did not meet SS-criteria. Her follow-up showed the detection of a nodule on the hard palate with the diagnosis of B-cell lymphoma based on morphologic and immunohistochemical staining (CD20+ B cells) from nodule biopsy. Gastrointestinal endoscopy showed chronic gastritis (Helicobacter pylori negative). The computerized tomography (CT) of neck, chest, abdomen, and pelvis, showed no additional evidence of disease. Recently a new episode of right parotitis along with a biopsied cervical lymph node confirmed persistent lymphoma after 6 months of rituximab.

A 16-year-old boy presented with intermittent painless cervical lymphadenopathy and bilateral parotid swelling for more than 5 years. Neck CT demonstrated enlargement of the parotids. A fine needle aspiration showed a benign lymphoid tissue with no evidence of malignancy. Laboratory data showed an increased ESR (122mm/h). Histopathology analysis showed infiltration of the salivary gland by a lymphocytic proliferation forming confluent nodular masses and follicular hyperplasia of the attached lymph nodes, indicative of a lymphoid malignancy (Fig. 1). Immunohistochemical stains demonstrated sheets of CD20+ B cells that were negative for CD5, CD43, and cyclin-D1, confirming B-cell clonal proliferation (Fig. 2). CD10 and BCL-6 stains highlighted germinal centers which were negative for BCL-2. A Ki-67 stain showed numerous positive cells within and between germinal centers (20–30% cells). Parotidectomy and radiotherapy was performed. Serologic testing revealed only a remote infection by CMV and EBV. Immunological tests showed positive RF, ANA, anti-SSA and anti-SSB antibodies. Despite only vague sicca symptoms, ophthalmology exam revealed corneal and conjunctival erosions, and the diagnosis of SS was made. Hydroxychloroquine and rituximab were started. There was no evidence of tumor recurrence after >14 months follow-up.

Fig. 1.

Biopsy of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the parotid gland in case 2. Left image shows Hematoxylin & Eosin (H&E) stain in low-power view. Right image shows H&E stain in high-power view.

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Fig. 2.

Biopsy of low grade B-cell lymphoma of MALT type of the parotid gland. CD 20 stain showing sheets of B cells in case 2.

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The authors declare that they have followed the protocols of their work centre on the publication of patient data.

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.