TY - JOUR
T1 - Locally aggressive and multifocal phosphaturic mesenchymal tumors
T2 - two unusual cases of tumor-induced osteomalacia
AU - Higley, Meghan
AU - Beckett, Brooke
AU - Schmahmann, Sandra
AU - Dacey, Elizabeth
AU - Foss, Erik
N1 - Publisher Copyright:
© 2015, ISS.
PY - 2015/12/1
Y1 - 2015/12/1
N2 - 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.
AB - 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.
KW - Fibroblast growth factor 23
KW - Oncogenic osteomalacia
KW - Phosphaturic mesenchymal tumor
KW - Tumor-induced osteomalacia
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U2 - 10.1007/s00256-015-2246-x
DO - 10.1007/s00256-015-2246-x
M3 - Article
C2 - 26341245
AN - SCOPUS:84944351928
SN - 0364-2348
VL - 44
SP - 1825
EP - 1831
JO - Skeletal Radiology
JF - Skeletal Radiology
IS - 12
ER -