A 60-year-old female with known Sjogren's syndrome presents with bilateral parotid enlargement, dry mouth, dry eyes, and an elevated serum anti-Ro (SS-A) antibody. Histopathology of a labial salivary gland biopsy shows focal lymphocytic sialadenitis with a focus score of ≥1. Which malignancy is she at significantly higher risk of developing compared to the general population?

• A Squamous cell carcinoma of the oral cavity
• B Adenoid cystic carcinoma of the submandibular gland
• C Mucosa-associated lymphoid tissue (MALT) lymphoma of the parotid ✓
• D Acinic cell carcinoma of the parotid

Explanation

Patients with primary Sjogren's syndrome have a 40-fold increased risk of developing MALT (mucosa-associated lymphoid tissue) lymphoma, typically arising in the parotid gland. Chronic antigenic stimulation of lymphocytes in the salivary glands leads to clonal B-cell expansion and eventual lymphomagenesis. Warning signs of MALT lymphoma in Sjogren's patients include sudden enlargement of a parotid swelling, palpable purpura, peripheral neuropathy, low C4 complement, and cryoglobulinemia. This is a clinically important and commonly tested fact.

Reference: Dhingra Diseases of Ear, Nose and Throat, 7th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.