The Clinical Approach Toward Giant Cell Tumor of Bone

• Published in: The oncologist (Dayton, Ohio) Vol. 19; no. 5; pp. 550 - 561
• Main Authors: Heijden, Lizz, Dijkstra, P.D. Sander, Sande, Michiel A.J., Kroep, Judith R., Nout, Remi A., Rijswijk, Carla S.P., Bovée, Judith V.M.G., Hogendoorn, Pancras C.W., Gelderblom, Hans
• Format: Journal Article
• Language: English
• Published: Durham, NC, USA AlphaMed Press 01.05.2014
• Subjects: Antibodies, Monoclonal, Humanized - therapeutic use; Bone Neoplasms - diagnostic imaging; Bone Neoplasms - drug therapy; Bone Neoplasms - pathology; Combined Modality Therapy; Curettage; Denosumab; Diphosphonates - therapeutic use; Female; GCTB; Giant cell tumor of bone; Giant Cell Tumor of Bone - diagnostic imaging; Giant Cell Tumor of Bone - drug therapy; Giant Cell Tumor of Bone - pathology; Histopathology; Humans; Magnetic Resonance Imaging; Male; Radiography; Radiotherapy; RANK ligand; RANK Ligand - antagonists & inhibitors; RANK Ligand - biosynthesis; Review; Sarcomas; Giant cell tumor of bone; Curettage; RANK ligand; Histopathology; GCTB; Review; Denosumab; Radiotherapy
• ISSN: 1083-7159; 1549-490X
• DOI: 10.1634/theoncologist.2013-0432

We provide an overview of imaging, histopathology, genetics, and multidisciplinary treatment of giant cell tumor of bone (GCTB), an intermediate, locally aggressive but rarely metastasizing tumor. Overexpression of receptor activator of nuclear factor κB ligand (RANKL) by mononuclear neoplastic stromal cells promotes recruitment of numerous reactive multinucleated giant cells. Conventional radiographs show a typical eccentric lytic lesion, mostly located in the meta‐epiphyseal area of long bones. GCTB may also arise in the axial skeleton and very occasionally in the small bones of hands and feet. Magnetic resonance imaging is necessary to evaluate the extent of GCTB within bone and surrounding soft tissues to plan a surgical approach. Curettage with local adjuvants is the preferred treatment. Recurrence rates after curettage with phenol and polymethylmethacrylate (PMMA; 8%–27%) or cryosurgery and PMMA (0%–20%) are comparable. Resection is indicated when joint salvage is not feasible (e.g., intra‐articular fracture with soft tissue component). Denosumab (RANKL inhibitor) blocks and bisphosphonates inhibit GCTB‐derived osteoclast resorption. With bisphosphonates, stabilization of local and metastatic disease has been reported, although level of evidence was low. Denosumab has been studied to a larger extent and seems to be effective in facilitating intralesional surgery after therapy. Denosumab was recently registered for unresectable disease. Moderate‐dose radiotherapy (40–55 Gy) is restricted to rare cases in which surgery would lead to unacceptable morbidity and RANKL inhibitors are contraindicated or unavailable. The authors provide an overview of imaging, histopathology, genetics, and multidisciplinary treatment of giant cell tumor of bone (GCTB), an intermediate, locally aggressive but rarely metastasizing tumor. This review addresses most relevant issues concerning diagnosis and multidisciplinary treatment of GCTB and future perspectives.