Hypophosphaturic mesenchymal tumor of the ethmoid associated with oncogenic osteomalacia.
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.
Ungari
Claudio
C
Department of Maxillofacial Surgery, University of Rome La Sapienza, Rome, Italy.
Rocchi
Giovanni
G
Rinna
Claudio
C
Agrillo
Alessandro
A
Lattanzi
Alessandro
A
Pagnoni
Mario
M
D000328:Adult; D005005:Ethmoid Sinus / Q000473:pathology*; D006801:Humans; D017674:Hypophosphatemia / Q000209:etiology* / Q000652:urine; D008297:Male; D008637:Mesenchymoma / Q000150:complications*; D009296:Nasal Cavity / Q000473:pathology; D009361:Neoplasm Invasiveness; D010018:Osteomalacia / Q000209:etiology*; D010255:Paranasal Sinus Neoplasms / Q000150:complications*; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000652:urine; D012279:Rickets / Q000209:etiology; D013101:Sphenoid Sinus / Q000473:pathology