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Hypophosphaturic mesenchymal tumor of the ethmoid associated with oncogenic osteomalacia
Authors:Ungari Claudio  Rocchi Giovanni  Rinna Claudio  Agrillo Alessandro  Lattanzi Alessandro  Pagnoni Mario
Affiliation:Department of Maxillofacial Surgery, University of Rome La Sapienza, Rome, Italy.
Abstract:
Oncogenic osteomalacia is an uncommon syndrome characterized by bone pain, proximal muscle weakness, hypophosphatemia, hyperphosphaturia, and a low plasma concentration of 1,25-dihydroxy-vitamin D. The disease affects both sexes at around 40 years of age, although it can sometimes affect children and adolescents. Generally, the syndrome is associated with a tumor, usually benign, of mesenchymal origin and is resolved after removal of the tumor; this syndrome can sometimes be associated with malignant tumors. These tumors seem to be histologically heterogeneous and are generally localized in soft tissues and bone. In this article, a case of oncogenic osteomalacia associated with a hypophosphaturic mesenchymal tumor of the ethmoid is reported in a 24-year-old man. After surgical and radical removal of the tumor, the patient noted a decrease in the clinical symptoms and signs.
Keywords:
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