A 50-year-old man has slowly progressive, bilateral, firm, slightly tender submandibular gland swellings. Biopsy shows dense lymphoplasmacytic infiltrate with storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are markedly elevated. What is the diagnosis and expected response to glucocorticoids?

• A Chronic nonspecific sialadenitis, no response to steroids
• B IgG4-related sclerosing sialadenitis, dramatic response to steroids ✓
• C Acinic cell carcinoma, partial response to radiotherapy
• D Sarcoid sialadenitis, response to antitubercular therapy

Explanation

This is Kuttner tumour, now classified as IgG4-related sclerosing sialadenitis, characteristically affecting the submandibular glands bilaterally. Histology shows a dense IgG4-positive plasma cell infiltrate with storiform fibrosis and obliterative phlebitis, and serum IgG4 is elevated. It responds dramatically to systemic glucocorticoids, distinguishing it from neoplastic causes. Antitubercular therapy is irrelevant here since granulomas and caseation are absent, and sarcoidosis typically involves the parotid rather than the submandibular glands.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.