Skip to main content

Advertisement

Table 1 Comparison of the characteristics of Cherubism and idiopathic CGCGs

From: Bilateral Central Giant Cell Granuloma of the mandibular angle in three females from the same family

  Cherubism Idiopathic CGCG
Aetiology Caused by a gain-of-function mutation in the gene coding a c-Abltyrosine kinase-binding protein (SH3BP2) located on the short arm of chromosome 4 The true aetiology is unknown and still controversial. It was thought that it is a reparative component. However, the evidence is not available to classify the lesions as reparative. The CGCG is thought by many to be reactive, but it is classified as a benign, non-neoplastic lesion.
Gender distribution More diffused in males (or equally diffused between males and females) More diffused in females
Age distribution More prevalently diagnosed in children CGCGs mainly affect patients between 10 and 30 years
Facial aspect Swelling of bilateral mandibular angle region, typical of Cherubism (accompanied by hypertelorism) Normal
Other signs A marked cervical lymphadenopathy is common. None
Definition (concept) Cherubism is an autosomal dominantly inherited condition, with variable expressivity, that is characterized by multi-quadrant radiolucent lesions of the jaws and a progressive and clinically, symmetrical enlargement of the mandible and/or the maxilla. Central giant cell granuloma (CGCG) is defined by the World Health Organization as an intraosseous lesion. The biologic behaviour ranges from quiescent to aggressive, with pain, root resorption and a tendency to recurrence after puberty.
Mandibular Lesions Symmetrical mandibular lesions Lesions are typically found unilaterally in the frontal region of the mandible. Sometime the lesion is located in a mandibular angle.
Family occurrence There is usually a familial history of similarly affected family members. Sometime they show an autosomal inheritance. In these cases, when bilateral, they are defined cherubism-like lesions.
Histological aspect of lesions The lesions appear microscopically generally indistinguishable from CGCG, except occasionally, when a fairly characteristic condensation of perivascular collagen is evident Cellular fibrous tissue that contains multiple foci of haemorrhage, aggregations of multiple nucleated giant cells, and occasionally trabeculae of woven bone.
Images and Rx aspects Multi-quadrant radiolucent lesions of the jaws At the Rx can be observed a marked displacement or agenesia of second and third molars as well as premature exfoliation of primary teeth. Osteolytic lesions of the jaw
Differential diagnosis Neurofibromatosis type 1, gingival fibromatosis as well as Noonan’s syndrome, all of them are Rasopathies Neurofibromatosis type 1, gingival fibromatosis as well as Noonan’s syndrome, all of them are Rasopathies
Treatments Treatment of lesions consists of local curettage, jaw contouring, intralesional steroid injections, and systemic calcitonin administration as well Commonly treated by surgical curettage.
Long-term clinical management Long-term follow-up Long-term follow-up
Prognosis The regression of the lesions is often seen following puberty These lesions tend to increase before the puberty (perhaps due to ovarian hormones) and to stabilize after puberty.