title: Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck.
abstract: Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that secretes fibroblast growth factor-23 (FGF-23) and causes oncogenic osteomalacia. It occurs in adults with equal gender distribution and the most common location is the lower extremities, followed by the head and neck. Besides osteomalacia, the clinical presentation includes bone pain and multiple bone fractures. Microscopic features consist of spindle cells, multinucleated giant cells, and calcifications embedded in a chondromyxoid matrix. Laboratory findings indicate normal calcium and parathyroid levels, hypophosphatemia, and increased levels of FGF-23 that usually revert to normal after surgical removal. Due to its rarity, the purpose of the study was to report 2 new oral cases of PMT and to review the literature in the head and neck. The first case occurred in the gingiva and had been present for 6 years. The second case was a recurrence of a previously diagnosed PMT in the right mandible that metastasized to the lung and soft tissue. The literature review included 53 cases in the head and neck. There was a predilection for extra-oral sites (76%) compared to intra-oral sites (24%) with paranasal sinuses considered the most common location (38%) followed by the mandible (15%). There were 9 recurrences that included 3 malignant cases indicating a potentially aggressive tumor. Due to the indeterminate biological behavior of PMT and its rarity, a comprehensive evaluation of medical, laboratory, radiographic, and histological findings are crucial for a definitive diagnosis and treatment.
authors.affiliation: Department of Diagnostic Sciences, Texas A&M University Baylor College of Dentistry, 3302 Gaston Avenue Room # 214, Dallas, TX, 75246, USA. hqari@bcd.tamhsc.edu.
authors.forename: Hiba
authors.initials: H
authors.lastname: Qari
authors.affiliation: Oral and Maxillofacial Surgery Division, Department of Surgery, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-9109, USA.
authors.forename: Aya
authors.initials: A
authors.lastname: Hamao-Sakamoto
authors.affiliation: Private Practice OMS, Austin, TX, USA.
authors.forename: Clay
authors.initials: C
authors.lastname: Fuselier
authors.affiliation: Department of Diagnostic Sciences, Texas A&M University Baylor College of Dentistry, 3302 Gaston Avenue Room # 214, Dallas, TX, 75246, USA.
authors.forename: Yi-Shing Lisa
authors.initials: YS
authors.lastname: Cheng
authors.affiliation: Department of Diagnostic Sciences, Texas A&M University Baylor College of Dentistry, 3302 Gaston Avenue Room # 214, Dallas, TX, 75246, USA.
authors.forename: Harvey
authors.initials: H
authors.lastname: Kessler
authors.affiliation: Department of Diagnostic Sciences, Texas A&M University Baylor College of Dentistry, 3302 Gaston Avenue Room # 214, Dallas, TX, 75246, USA.
authors.forename: John
authors.initials: J
authors.lastname: Wright
mesh_terms: D000089703:Fibroblast Growth Factor-23; D005887:Gingival Neoplasms / Q000150:complications / Q000473:pathology; D006258:Head and Neck Neoplasms / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D008339:Mandibular Neoplasms / Q000150:complications / Q000473:pathology; D008637:Mesenchymoma / Q000150:complications / Q000473:pathology*; D008875:Middle Aged; D009372:Neoplasms, Connective Tissue / Q000209:etiology; D010018:Osteomalacia; D010257:Paraneoplastic Syndromes
keywords: Calcium; Hyperphosphaturia; Hypophosphatemia; Oncogenic osteomalacia; Phosphaturic mesenchymal tumor