173. Aggressive Cherubism: When is Surgery the Right Choice?

PURPOSE: Cherubism is a rare genetic disorder characterized by bilateral proliferative fibroossesous lesions of the mandible and maxilla, which may result in varying degrees of facial deformity, dental abnormalities, and functional impairments. While conservative management is commonly favored due to potential post-pubertal regression of lesions, data on surgical management for cases of aggressive cherubism is limited. This study reviews a single institution’s experience managing severe cherubism, focusing on surgical strategies to address significant functional and aesthetic concerns. We aim to provide insights into effective management of cherubism’s aggressive variants. METHODS: A retrospective chart review of patients diagnosed with cherubism at a single institution between 1997 and 2024 was conducted. Clinical presentation, as well as radiographic, histopathologic and/or genetic correlation were documented. Patients were graded from I-V using Motamedi’s classification,1 with Grade V being the most severe. Data on symptoms, treatments, and complications was recorded. Outcomes were assessed based on symptom resolution, improvement in function, and aesthetic results. RESULTS: Nine patients (seven male, two female) met inclusion criteria, with a mean age at consultation of 8.1±3.4 years. All patients had mandibular involvement, while six also had maxillary involvement. Severity grades included: I (n=1), II (n=3), III (n=2), IV (n=1), V (n=2). All nine patients presented with bilateral facial swelling and jaw deformity, and eight had dental abnormalities. Both Grade V patients presented with respiratory symptoms and proptosis, and one had orbital compression and hyponasal speech. Seven of nine (77.8%) patients underwent surgical resection. The two Grade V patients underwent multiple resections (four and six, respectively), and one received adjuvant denosumab therapy. The mean age at initial resection was 10.1±4.3 years and the average resected tumor volume was 79.2±50.9 cm³. Surgical complications included temporary bilateral V3 anesthesia and mandibular abscess. Surgical outcomes were favorable, with improved facial symmetry in all treated patients and significant improvement in proptosis and respiratory symptoms in Grade V patients. CONCLUSION: These findings indicate that surgical management is an effective option for patients with cherubism presenting with rapidly expanding lesions, severe asymmetry, or impending pathologic fractures, offering improved functional and aesthetic outcomes with a low complication profile. Severe cases with symptoms such as orbital compression and respiratory distress necessitated multiple resections and adjunctive pharmacologic therapy, highlighting the need for individualized treatment plans, particularly for patients with Grade V cherubism. 1. Motamedi MHK. Treatment of cherubism with locally aggressive behavior presenting in adulthood: report of four cases and a proposed new grading system. J Oral Maxillofac Surg 1998;56:1336-42.