Unique Experience of Cherubism Targeted Therapy

The family form of giant cell reparative granuloma or cherubism is a rare benign lesion of the jaws which causes face deformation reminiscent of the cherubs portrayed in Renaissance art. Radical surgery, especially in children before puberty, is impossible or irrational, because it leads to disablement. For four years, a child with cherubism was undergoing an outpatient supervision in the Department of Maxillofacial Surgery in Russian Children Clinical Hospital. During the observation period it was noted that tumor masses were slowly progressing leading to exoorbitism. The child had a diagnosis histological verification; a treatment using human monoclonal antibodies to RANKL was developed, adapted and approved by the ethical committee. The clinical effect was observed from the third month of therapy - jaws corners sharpened, the volume reduced. The control biopsy material taken at the end of the 6-month course did not reveal any giant cells which meant complete pathomorphism. Computed tomography showed that at the end of the therapy bone density increased by 6-7 times in the affected zones and was additionally growing over the next 6 months of observation. Such dynamics allowed making effective contouring surgery of excessive bone tissues. Inoperable and early stages of cherubism require a combined treatment which includes a course of monoclonal antibodies followed by alendronic acid therapy that increases osteoblasts survival. After the treatment, if it is necessary to improve the appearance, contouring surgery of excessive bone tissue is performed.

Authors
Kugushev A.Y.1 , Lopatin A.V. 1, 2 , Yasonov S.A. 1
Number of issue
Issue
Language
Russian
Pages
608-622
Status
Published
Volume
27
Year
2019
Organizations
  • 1 Russian Children's Clinical Hospital of Pirogov Russian National Research Medical UniversityMoscow 119571, Russian Federation
  • 2 RUDN UniversityMoscow 117198, Russian Federation
Keywords
alendronic acid; bisphosphonates; cherubism; children; denosumab; giant cell reparative granuloma
Date of creation
24.12.2019
Date of change
24.12.2019
Short link
https://repository.rudn.ru/en/records/article/record/55112/
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