Noonan-like Syndrome with Multiple Giant Cell Lesions in the Jaws: A Case Report

Abstract

Aims: To present a case report of a patient with Noonan syndrome who presented with multiple giant cell lesions in the mandible and describe the management and follow-up of this pathology, discussing the different treatment modalities and our experience. Presentation of case: We present the case of a 7-year-old boy with Noonan phenotype and genetic SOS1 mutation who presented with bilateral giant cell lesions of the mandible. He underwent curettage and steroid injection, presenting with recurrence in the follow-up. Corticosteroid injections were decided given the small size of the lesion with good results. However, 2 years later, he presented with a new recurrence that required a new curettage. He is now free of recurrence, with satisfactory bone regeneration and occlusion. Discussion: Giant cell lesions are frequently found in Noonan syndrome patients. They are usually multiple and indolent but can show an aggressive behaviour. Different treatment modalities have been described in the management of giant cell lesions. Curettage is the most frequently used. Nonetheless, reports of high recurrence with this technique have been made in the literature and several authors have applied more aggressive surgical resections, with the high morbidity it entails. Moreover, several adjunctive treatments have also been described, such as corticosteroid injections, interpheron alpha-2a, calcitonin, etc. with variable and unclear results. Conclusion: We believe the combination of corticosteroids and surgical curettage is an adequate treatment since it can lower recurrence rates and minimizes severity and morbidity while preserving the dentition and mandibular morphology.