[Bilateral giant cell central granuloma of the jaws in a Noonan syndrome: About one case with emphasizing on bone giant cell lesions of the jaws]

Abstract

We report the case of a 10-year-old child with bilateral mandibular localization of a central giant cell granuloma occurring in the setting of Noonan syndrome. The histological appearance was classic with two intermigled components, one fibrous with non-atypical mononuclear cells, the other consisting of numerous osteoclast-like giant cells. This aspect is similar to that observed in the brown tumor as well as that of cherubism, which can also give multiple bone lesions. We will discuss the other lesions to consider in case of benign giant cell bone lesions affecting the jawbones, sometimes multiple and part of which falls within the scope of RASopathies.

Keywords: Brown tumor; Central giant cell granuloma of the jaws; Cherubism; Chérubisme; Granulome central à cellules géantes des mâchoires; Hyperparathyroidism; Hyperparathyroïdisme; Multifocality; Multifocalité; Noonan syndrome; RASopathies; Syndrome de Noonan; Tumeur brune.