Non-Hodgkin lymphoma and Sjogren's syndrome

Case contributed by James Sheldon
Diagnosis certain

Presentation

Right facial lump

Patient Data

Age: 75 years
Gender: Male

Numerous small cysts are identified within the parotid glands bilaterally, the appearance of which is typical for Sjogren syndrome/disease.

In the anterior aspect of the right parotid gland a more heterogeneous mass is identified.

No evidence of perineural spread of tumor.

No lymph node enlargement in the field of view.

Core biopsy of the right parotid mass has been performed.

Case Discussion

Patients with Sjogren disease have a considerably increased risk of developing Non-Hodgkin lymphoma (approx 10 x higher than the general population).

Typical imaging appearance of Sjogren disease in the parotid glands involves numerous small cysts. The development of a mass within the parotid glands is highly concerning for NHL.

Histopathology:

MICROSCOPIC EXAMINATION: The sections from this small fragmented salivary gland tissue shows diffuse proliferation of lymphocytes in areas of lymphoepithelial lesions. The lymphocytes are small to medium sized with ovoid to mildly cleaved nuclei, small amount of sometimes clear cytoplasm and low mitotic count. There is background population of small reactive lymphocytes.

IMMUNOPHENOTYPING: CD20/Pax-5: Strongly positive CD3/5: Large numbers of positive cells consistent with reactive T-cells, CD23: Dendritic staining shows follicles and follicle remnants, CD10: Focal staining in follicle remnants only, Bcl-6: Positive, patchy, Bcl-2: Diffusely strongly positive, Ki-67: 20%. 

CONCLUSION: The features are those of a low-grade B-cell non-Hodgkin lymphoma, with immunohistochemistry profile consistent with marginal zone lymphoma.

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