[Unique Experience of Cherubism Targeted Therapy]

Area of Science:

• Maxillofacial Surgery
• Pediatric Oncology
• Genetics

Background:

• Cherubism is a rare, benign jaw lesion causing significant facial deformity.
• Surgical intervention in children is often contraindicated due to potential disablement.
• Progression of cherubism can lead to complications like exophthalmos.

Purpose of the Study:

• To evaluate the efficacy of human monoclonal antibodies to RANKL in treating cherubism.
• To assess the impact of this therapy on bone density and lesion volume.
• To establish a combined treatment protocol for cherubism.

Main Methods:

• Histological verification of cherubism diagnosis.
• Treatment with human monoclonal antibodies to RANKL over a 6-month course.
• Monitoring clinical effects, including lesion volume and bone density via CT scans.
• Follow-up biopsies to assess histological changes.

Main Results:

• Significant reduction in jaw lesion volume observed from the third month of therapy.
• Complete pathomorphism achieved, with no giant cells detected in follow-up biopsies.
• Bone density in affected areas increased 6-7 times post-therapy, with continued growth.
• Effective contouring surgery became feasible after the combined treatment.

Conclusions:

• Human monoclonal antibodies to RANKL represent a viable therapeutic option for cherubism, particularly in pediatric cases.
• A combined treatment approach, including monoclonal antibodies and alendronic acid, followed by contouring surgery, offers a comprehensive management strategy.
• This non-surgical approach mitigates the risks associated with radical surgery in early-stage cherubism.