Abstract
Giant cell tumors (GCTs) and central giant cell granulomas (CGCGs) are aggressive lesions that appear in the jaw. These lesions occur in the second and third decades of life and often arise in the mandible. Clinical manifestations of these lesions vary from asymptomatic to symptomatic tooth displacement with cortical perforation. GCTs, which are characterized by multinucleated osteoclast‐type giant cells that express receptor activator of nuclear factor‐κB (RANK) ligand, rarely present in the jaw and have overlapping histopathologic features with CGCGs, which are composed of fibroblastic stromal cell lesions. GCTs and CGCGs have overlying histopathologic features that make distinction between the two challenging. There is a real controversy as to whether giant cell tumors and central giant cell granulomas are in fact, one and the same lesion. The majority of GCTs occur in the long bone, with surgery being the typical therapeutic option. Denosumab as a treatment modality is a fairly new concept that has been used effectively in GCTs affecting long bones. There is less experience, however, with its use for jaw lesions. This seven‐case series describes the effective use of both low‐dose and high‐dose denosumab in the treatment of GCTs and CGCGs affecting the jaw and special dosing considerations for younger patients who present with disease. © 2017 The Authors. JBMR Plus Published by Wiley Periodicals, Inc. on behalf of the American Society for Bone and Mineral Research.
Highlights
Giant cell tumors (GCTs) and central giant cell granulomas (CGCGs) are similar appearing lesions that appear in the jaw
Tooth displacement and expansion with cortical perforation may occur in more aggressive lesions.(1) GCTs of bone, generally considered a separate entity from CGCGs, are lesions characterized by multinucleated osteoclast-type giant cells that express receptor activator of nuclear factor-kB (RANK) and mononuclear stromal cells that express RANK-ligand (RANKL).(2) GCTs are rarely present in the jaws but have overlapping histopathologic features with CGCG that make distinction between the two lesions challenging
The giant cells in CGCGs are CD68-positive and vary in size, shape, and number.(1) Comparatively, histologic features that favor the diagnosis of GCT over CGCG include sheets of neoplastic ovoid mononuclear cells with high RANKL expression, RANKpositive mononuclear cells of myeloid lineage, and large RANKexpressing osteoclast-like giant cells.(3) there has been a distinct delineation between GCTs and CGCGs based on the aggressive behaviors of GCTs, clinical and histomorphologic data suggest that GCT and CGCG represent a spectrum of a single disease process modified by patient age and site of lesion occurrence.(4)
Summary
Giant cell tumors (GCTs) and central giant cell granulomas (CGCGs) are similar appearing lesions that appear in the jaw. Tooth displacement and expansion with cortical perforation may occur in more aggressive lesions.(1) GCTs of bone, generally considered a separate entity from CGCGs, are lesions characterized by multinucleated osteoclast-type giant cells that express receptor activator of nuclear factor-kB (RANK) and mononuclear stromal cells that express RANK-ligand (RANKL).(2) GCTs are rarely present in the jaws but have overlapping histopathologic features with CGCG that make distinction between the two lesions challenging. We present a case series of one GCT of the jaw, five CGCG of the jaw, and one case of giant cell lesion consistent with cherubism, all of which were referred to endocrinology to determine whether medical therapy could obviate the need for planned surgical resection. Our primary endpoint in this series was to identify whether surgical
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