Bilateral parotid gland MALT lymphoma in a patient with Sjögren’s and sicca syndrome

Findings

CT Neck: Enlarged and cystic change of the bilateral parotid glands and submandibular glands without discrete enhancing mass.
MR neck: Diffuse cystic replacement and enlargement of the bilateral parotid glands (and to a lesser degree submandibular glands). No discreet enhancing parotid mass identified.

Differential diagnosis
- Non-Hodgkin's lymphoma parotid
- Benign lymphoepithelial lesions – HIV (BLL-HIV)
- Sarcoid
- Warthin tumor


Diagnosis: Bilateral parotid gland MALT lymphoma in a patient with Sjögren’s and sicca syndrome.


Key Points

Sjögren's syndrome is the second most common autoimmune disease after rheumatoid arthritis.
Sjögren's syndrome is a chronic autoimmune exocrinopathy causing salivary and lacrimal gland destruction.
Clinical: Recurrent acute episodes of glandular swelling (and dry eye, mouth, skin).
CT findings: Parotid enlargement, numerous cystic lesions, +/- punctuate calcifications
MR findings: Diffuse cystic lesions (T1 hypo, T2 hyper); heterogeneous mild enhancement of nodular parenchyma and fibrosis on T1+C, with non-enhancing cysts
Appearance of cysts helps in staging: Cysts 1-2 mm suggest Stage I or II disease. Larger cysts of > 2mm suggest Stage III, IV
Stages II-IV are readily seen on parotid US
Non-Hodgkin's lymphoma frequently complicates chronic Sjögren's parotid involvement
Bilateral parotidectomy in this patient was performed for recurrent infection / intractable pain and the surgical pathology showed bilateral MALT lymphoma