Bone Abstracts

Introduction: Bone metastasis pathophysiology is currently defined as a vicious circle. Indeed, tumor cells co-express RANK-L and its receptor, RANK, allowing their own proliferation and peritumoral bone resorption necessary to the lesion expansion. Odontogenic tumors, which occur within the jaw, seem to present the same pathophysiological characteristics. Thus, studying the impact on bone of RANK over-expression by the osteoclasts would permit a better understanding of those ...

Bisphosphonates (BP) are powerful bone resorption inhibitors. They are used for the symptomatic treatment of malignant osteolytic bone disease (e.g. multiple myeloma and bone metastasis), as well as bone diseases associated with high bone resorption (e.g. postmenopausal osteoporosis, cortisone-induced osteoporosis). However, recent data showed that a rare, but serious, adverse effect of BP therapy is osteonecrosis of the jaw (BRONJ). Given the increasing number of persons rece...

Introduction: Cherubism is a rare pediatric disease with a maxillofacial localization caused by mutations of the SH3BP2 gene. Pathogenesis is well described in the Sh3bp2 KI mouse model that presents a systemic inflammatory and bone phenotypes maintained by TNFα and due to the presence of hypersensitive myeloid precursors. In human, the disease is usually described as a maxillofacial exclusive disease. The aim of our study was to explore the systemic phe...

Cherubism is a rare genetic disease (OMIM #118400) characterized by a massive jaw bone osteolysis. This pathology appears around 2–5 years old and in the less severe cases spontaneously regresses after puberty. So far the only treatment available is surgery, often disabling and traumatic. As the cherubism pathophysiology is not yet understood, we carried out a thoroughly characterization of the cherubism granulomas from ten unrelated patients to determine the cells involv...

Cherubism is a rare genetic disorder characterized by extensive growth of a bilateral granuloma of the jaws, resulting in facial disfigurement.Histologically, the lesions consist of a fibrotic stroma with osteoclastic-like multinuclear giant cells (MGC). Cherubism is caused by gain-of-function mutations in the SH3BP2 protein. SH3BP2 is an intracellular adaptor protein positively regulating the activity of the nuclear factor of activated T-cells c1 (NFATc...