A young man evaluated for recurrent headaches has a CT showing a dense, well-circumscribed bony mass arising from the frontal sinus. On further evaluation he is found to have multiple desmoid tumours and colonic polyposis. This association is known as:

• A Gardner syndrome ✓
• B Gorlin-Goltz syndrome
• C McCune-Albright syndrome
• D Cowden syndrome

Explanation

Osteoma is the most common benign tumour of the nose and paranasal sinuses, favouring the frontal and ethmoid sinuses. Multiple osteomas occurring with intestinal polyposis, epidermal cysts, fibromas, and desmoid tumours define Gardner syndrome, an APC gene-related condition carrying high colorectal cancer risk. Gorlin-Goltz features multiple odontogenic keratocysts and bifid ribs, while McCune-Albright features polyostotic fibrous dysplasia with cafe-au-lait spots.

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.