CLINICAL Q&A

Clinical Q+A in Oral Medicine

Answer

ANSWER

Salivary gland neoplasms Bony pathology Immune-mediated conditions

Differential diagnosis for this presentation includes neoplastic processes including salivary gland neoplasms (pleomorphic adenoma, Warthin’s tumour, mucoepidermoid carcinoma); metastatic neoplasm (cutaneous squamous cell carcinoma metastasised to parotid gland); bony pathology (odontogenic keratocyst, ameloblastoma); immune mediated conditions (Sjogren’s syndrome with associated lymphoma, sarcoidosis); and to a lesser extent an infectious condition including mumps, EpsteinBarr virus, tuberculosis or humanimmunodeficiency virus.

WHAT INVESTIGATIONS WOULD ASSIST IN COMING TO THE DEFINITIVE DIAGNOSIS?

• CT head and neck with contrast • Fine needle biopsy/core biopsy

INVESTIGATIONS

I organised a CT head and neck with contrast for the patient. The CT demonstrated a large (48mm) slightly ill-defined mass arising within the anterior aspect of the superficial lobe of the left parotid gland (Figure 2). There was generalised atrophy of the lacrimal glands, right parotid gland and both submandibular salivary glands that are strongly suggestive of Sjogren’s Syndrome. Blood investigations were also strongly positive for SS-A, SS-B and SSA Ro52 extractable nuclear antigens. Rheumatoid factor (RF) was borderline positive. The patient underwent a core biopsy of the left parotid mass, that confirmed a diffuse large B-cell lymphoma. The final diagnosis was confirmed as Stage II E diffuse large B cell lymphoma (DLBCL) (AJCC 8th edition) associated with primary Sjogren’s Syndrome. I subsequently referred the patient to haematology colleagues where he underwent chemotherapy for the management of DLBCL with R-CHOP (rituximab, cyclophosphamide, vincristine, prednisolone). Of all the systemic autoimmune diseases, Sjogren’s Syndrome (SS) has the highest risk of lymphoma development. Approximately 5-10% of SS patients develop lymphoma, with risk of development increasing with duration of SS. Salivary gland enlargement, lymphadenopathy, Raynaud’s phenomenon, Anti-SSA/Ro and/or anti-SSB/La positivity, rheumatoid factor positivity, monoclonal gammopathy and C4 hypocomplementemia are independent risk factors for lymphoma development among SS patients. The risk increased to almost 40% in patients displaying three to six risk factors and increases to 100% if all seven risk factors are present. The above case highlights the complications that can occur with Sjogren’s Syndrome. It is essential to recognise and follow-up on independent risk factors for lymphoma development from the initial diagnosis of SS, in order to recognise high-risk patients.

Dr Lalima Tiwari

Oral medicine specialist Perth Oral Medicine and Dental Sleep Centre

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