Denosumab for the Treatment of Aggressive Recurrent Central Giant Cell Granuloma of the Maxilla in a Patient with Osteoglophonic Dysplasia.

Osteoglophonic dysplasia (OD) is a rare skeletal disorder marked by craniosynostosis, craniofacial anomalies, and abnormal bone development, primarily caused by FGFR gene mutations. We present the case of a 26-year-old Chinese female, who has been followed since age 11 for recurrent central giant cell granulomas (CGCG) and non-ossifying fibroma affecting her jaws and lower limbs. While her craniofacial and limb features have been described in two earlier publications, this report provides a detailed account of the aggressive and recurrent maxillary CGCG that resulted in significant clinical morbidity. Despite treatment with Alendronate targeting femoral lesions, the patient sustained a pathological fracture, resulting in long-term wheelchair dependency. Additionally, the maxillary lesion persisted and extended into the ethmoid sinus, causing recurrent epistaxis and anemia that required multiple surgical interventions. A multidisciplinary team initiated treatment with Denosumab, a monoclonal antibody that inhibits receptor activator of nuclear factor-κB ligand (RANKL) and directly targets osteoclast-like giant cells. Compared to bisphosphonates like Alendronate, which reduce bone resorption indirectly, Denosumab acts more selectively and showed superior clinical response in this case. The therapeutic switch resulted in marked tumor regression, effectively reducing surgical burden and improving quality of life. This case highlights the complexity of managing OD-associated CGCGs and the potential of Denosumab as a more effective therapeutic option than Alendronate. However, ongoing surveillance for adverse effects such as medication-related osteonecrosis of the jaw, hypocalcemia, and atypical fractures is essential.