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Locally aggressive and multifocal phosphaturic mesenchymal tumors: two unusual cases of tumor-induced osteomalacia.

Abstract
Tumor-induced osteomalacia (TIO) has long been recognized as a clinical paraneoplastic syndrome. The identification of a unique histopathologic entity, the phosphaturic mesenchymal tumor (PMT), as a distinct etiology for TIO has been a more recent discovery. The majority of published cases describe a solitary, non-aggressive appearing soft tissue or osseous lesions in patients with osteomalacia; aggressive appearing or multifocal lesions appear to be exceedingly rare. These tumors characteristically secrete fibroblast growth factor 23 (FGF23). Elevated serum levels of FGF23 result in phosphate wasting and osteomalacia. In the majority of cases, laboratory abnormalities and clinical signs and symptoms of osteomalacia precede identification of the causative lesion by years. Following diagnosis, complete resection with wide margins to prevent local recurrence is most often curative. Imaging characteristics of PMT are diverse and remain incompletely defined, as the majority of previous publications are outside of the radiologic literature. We present multiple imaging modalities in two cases of patients with debilitating osteomalacia and unusual appearing PMTs: one with a locally aggressive lesion leading to pathologic fracture, the second presenting with exceedingly rare multifocal PMT.
AuthorsMeghan Higley, Brooke Beckett, Sandra Schmahmann, Elizabeth Dacey, Erik Foss
JournalSkeletal radiology (Skeletal Radiol) Vol. 44 Issue 12 Pg. 1825-31 (Dec 2015) ISSN: 1432-2161 [Electronic] Germany
PMID26341245 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Biomarkers, Tumor
  • FGF23 protein, human
  • Phosphorus
  • Fibroblast Growth Factors
  • Fibroblast Growth Factor-23
Topics
  • Biomarkers, Tumor (blood)
  • Diagnosis, Differential
  • Female
  • Fibroblast Growth Factor-23
  • Fibroblast Growth Factors (blood)
  • Humans
  • Middle Aged
  • Neoplasm Invasiveness
  • Osteomalacia (blood, diagnosis, etiology)
  • Paraneoplastic Syndromes (blood, diagnosis)
  • Phosphorus (blood)
  • Soft Tissue Neoplasms (blood, pathology)

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