Figure 3

Noonan/multiple giant cell lesion syndrome. A 20-year old woman with Noonan/multiple giant cell lesion syndrome with bilateral involvement of the mandible and the maxilla. A and B. Frontal and submental photographs reveal prominent maxillary and mandibular contours, slight frontal bossing and increased intercanthal distance, downslanting palpebral features, epicanthal folds and posteriorly angulated ears. Lesions were painless to palpation. C and D. Coronal and 3D CT scan at initial presentation demonstrate involvement of the maxilla, including the maxillary sinuses and the nasal-maxillary region and the mandible from the antegonial notch to antegonial notch. The lesions are expansile and mixed radiolucent - radiopaque with cortical thinning and expansion into the orbits bilaterally. Patient was positive for a Noonan syndrome mutation in SOS-1. She underwent a contour resection of the maxillary and the mandibular giant cell lesions in two sessions. Postoperatively, she was treated with 4 courses of zoledronic acid, given at 6-week-intervals. E and F. Post-treatment frontal and submental photographs.