Imaging characteristics of phosphaturic mesenchymal tumors

Acta Radiol. 2023 Jun;64(6):2061-2073. doi: 10.1177/02841851231160086. Epub 2023 Mar 13.

Abstract

Background: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome associated with phosphaturic mesenchymal tumors (PMTs). Localization of the causative tumor in these cases is an arduous task since the culprit lesions are usually small, slow-growing, and can be located almost anywhere from head to toe.

Purpose: To describe the morphological characteristics of histologically proven PMTs on various radiological modalities.

Material and methods: After institutional ethical approval, this retrospective study analyzed 20 cases with a histopathological evidence of PMT. Various imaging characteristics of the tumors on available computed tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated. Descriptive statistical analyses were conducted.

Results: The tumors were located in diverse locations: lower extremities (n = 10); head and neck (n = 5); vertebral column (n = 3); pelvis (n = 1); and upper extremities (n = 1). Bone lesions seen on CT had variable morphology: sclerotic (n = 3/8, 37.5%); lytic (n = 3/8, 37.5%), and both lytic and sclerotic (n = 2/8, 25%) with presence of narrow zone of transition in all cases (n = 8/8) and amorphous internal matrix calcifications in 25% of cases (n = 2/8). Of the tumors, 68.4% (n = 13/19) were hypointense on T1 and all of them showed hyperintense signal on T2-weighted and STIR images (n = 19/19) and contrast enhancement (n = 16/16). Of the tumors, 66.7% (n = 6/9) showed restricted diffusion. DOTANOC PET/CT showed tumor uptake in all cases (n = 8/8).

Conclusion: PMTs may have variable and non-specific tumor appearances on various imaging modalities. However, in an appropriate clinical scenario and a background of suggestive biochemical work-up, the radiologist should keep a high index of suspicion.

Keywords: Oncogenic osteomalacia; fibroblast growth factor; imaging; oncology; phosphaturic mesenchymal tumors.

MeSH terms

  • Humans
  • Paraneoplastic Syndromes* / complications
  • Paraneoplastic Syndromes* / diagnostic imaging
  • Paraneoplastic Syndromes* / pathology
  • Positron Emission Tomography Computed Tomography / methods
  • Retrospective Studies
  • Soft Tissue Neoplasms* / pathology
  • Tomography, X-Ray Computed / methods