Craniomaxillofacial Peripheral Osteomas in Gardner Syndrome

Gardner syndrome is a precancerous condition featuring familial adenomatous polyposis (FAP), multiple osteomas, and soft tissue tumors. Craniomaxillofacial peripheral osteomas often cause visible facial deformities, enabling early detection of the syndrome-frequently before FAP manifests-thus providing a critical window for intervention to prevent malignant transformation and reduce mortality. A systematic review searched 8 electronic databases from inception to December 2024. This was supplemented by one institutional case of a 21-year-old female with multiple craniomaxillofacial peripheral osteomas, who underwent successful surgical excision at age 26 and remained recurrence-free after 4 years of follow-up. Studies with confirmed craniomaxillofacial peripheral osteomas linked to Gardner syndrome were included. Data on epidemiology, clinical/radiologic features, and treatment were extracted. Pooled analysis of 113 cases (87 published studies plus one new case) showed these osteomas predominantly affect young patients (median age: 20 y). Lesions are typically multiple, located in the skull, paranasal sinuses, and mandible, causing noticeable cosmetic deformities. Imaging (plain radiographs, panoramic views, CT) reveals well-defined, round or oval radiopaque masses. Surgical excision is the main treatment for symptomatic or disfiguring lesions, with rare recurrence. Early recognition of these characteristic osteomas facilitates prompt FAP surveillance, enabling timely preventive measures that significantly improve outcomes and reduce mortality.