Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature.
Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome that results in renal phosphate wasting with hypophosphatemia. In most cases, the underlying cause of TIO is a small mesenchymal neoplasm that is often difficult to detect, resulting in delayed diagnosis. One such neoplasm is the phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT), an unusual entity with unique morphologic and biochemical features. Most of these tumors are found at appendicular sites with only rare cases reported in the jaws. We describe a PMTMCT involving the mandible in a patient with a protracted history of osteomalacia. A review of the current literature is provided with emphasis on the clinical and histologic features, etiopathogenesis, and management of PMTMCT in the setting of TIO.
Woo
Victoria L
VL
Department of Biomedical Sciences, School of Dental Medicine, University of Nevada, Las Vegas, NV 89106, USA. victoria.woo@unlv.edu
Landesberg
Regina
R
Imel
Erik A
EA
Singer
Steven R
SR
Folpe
Andrew L
AL
Econs
Michael J
MJ
Kim
Taeyun
T
Harik
Lara R
LR
Jacobs
Thomas P
TP
D000328:Adult; D005260:Female; D006801:Humans; D017674:Hypophosphatemia / Q000209:etiology / Q000473:pathology / Q000628:therapy; D008339:Mandibular Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D008637:Mesenchymoma / Q000150:complications* / Q000473:pathology / Q000601:surgery; D009372:Neoplasms, Connective Tissue / Q000150:complications* / Q000473:pathology / Q000601:surgery; D010018:Osteomalacia / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D010257:Paraneoplastic Syndromes / Q000150:complications / Q000473:pathology* / Q000628:therapy