Kugushev A.Yu., Lopatin A.V., Rogozhin D.V., Grachev N.S. Efficiency and safety of using monoclonal antibodies in the treatment of cherubism , analys is of 6 clinical cases . Head and neck . Russ i a n Journal. 2022;10(2):49–59 (In Russian).

Doi: 10.25792/HN.2022.10.2.49–59

Introduction: Juvenile giant cell reparative granuloma, or cherubism, is a rare benign lesion of the skull bones characterized by a total involvement of the upper and lower jaws with facial deformities similar to those in the Renaissance painting cherubs. Radical surgery, especially in prepubertal children, is either impossible or irrational to perform, as it leads to serious disability.
Materials and methods: For the period from 2016 to 2021, 6 children, aged 5 to 9 years and having grade 3 — 4 jaw involvement, have received a course of monoclonal antibodies for 6 months in the Department of Craniomaxillofacial
Surgery of the Russian Children’s Clinical Hospital. In all cases, there was a slowly progressive increase in jaw deformity, which in one case led to exophthalmos. After histological verification of the diagnosis, therapy with monoclonal antibodies was started with an individual selection of the dose based on weight-growth parameters.
Denosumab was injected subcutaneously at days 0, 8, and 15, and every 4 weeks for 6 months subsequently.
Results: The clinical effect has been noted since the third month — sharpening of the angles of the jaws, a decrease in their volume. A control assessment of biopsy material obtained at the end of a 6-month-long course of treatment
revealed complete response in all cases. The computed tomography data showed an increase in bone density from 70 to 600HU at the end of the treatment course with an additional increase during the subsequent 6 months of observation. Such dynamics made it possible to perform contour resection of excess bone tissue, which was required in 2 cases, safely and effectively.
Conclusion: For extensive and aggressive early onset cherubism, therapy with monoclonal antibodies allows to stop the pathological process and restore the affected area if all lytic areas are filled by the end of the treatment.
However, due to the risk of developing severe side effect on calcium-phosphorus metabolism, treatment should be supervised by a multidisciplinary team.
Key words: giant cell reparative granuloma, cherubism, denosumab, children, alendronic acid, bisphosphonates
Conflicts of interest. The authors have no conflicts of interest to declare.
Funding. There was no funding for this study.

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