Clinical Characteristics and Evolution of Giant Cell Tumor Occurring in Paget's Disease of Bone

• DOI: 10.1002/jbmr.2349
• Publication Date: 2014-09-05T03:11:57Z
• Authors: Domenico Rendina; Gianpaolo De Filippo; Stuart H Ralston; Daniela Merlotti; Fernando Gianfran...; Teresa Esposito; Riccardo Muscariello; Ranuccio Nuti; Pasquale Strazzullo; Luigi Gennari
• Keywords: Bone disease

ABSTRACT Patients with Paget's bone disease (PDB) have an increased risk of developing giant cell tumor (GCT). This study was performed to evaluate the clinical characteristics and evolution GCT complicating PDB compare these those observed in two large cohorts, Italian Registry United Kingdom's Multi-Centre Randomised Controlled Trial Symptomatic Versus Intensive Bisphosphonate Therapy for Disease (PRISM) study. A systematic literature review identified 117 cases complicated by (PDB-GCT), which involved skeletal sites affected (110 patients) or extraskeletal tissues adjacent bones (7 patients). In contrast what previously reported patients without (83.2%) were white one-fourth them (24.8%) had multifocal GCTs. Compared GCT, PDB-GCT showed a higher male/female ratio (2.1 versus 1.2) more severe (age at onset 52.1 ± 12.1 63.3 10.6 years; number 6.1 2.9 2.34 1.6; prevalence polyostotic 93.3% 60.6%). The mortality rate than occurring (about 50% 0% 5% 5 years) (log rank = 29.002). Moreover, up 98% elevated total alkaline phosphatase levels neoplasm diagnosis, suggestive active PDB. Importantly, from Southern Italy (45.6% all (51.7%) positive familial history (70.8%) (65.0%). Finally, indirect evidence suggests decline incidence patients. occurrence is associated reduced life expectancy diseases founder effect. changes over time could be related improved management and/or living conditions © 2014 American Society Bone Mineral Research.