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Background <p>To review and study 14 cases of phosphaturic mesenchymal tumor (PMT) patients, integrating previous literature to expand understanding of this disease, improve diagnosis rates, and enhance treatment efficacy.</p> Methods <p>This study included 14 patients with PMT who were treated and pathologically confirmed at our bone tumor center between December 2016 and September 2023. Clinical data, including imaging, pathological findings, and laboratory results, were collected and analyzed. All patients underwent preoperative PET/CT scanning, followed by surgery, and were monitored during an average postoperative follow-up period of 28.86 ± 21.72 months. The surgical procedures included tumor resection, curettage, and ablation.</p> Results <p>The study cohort comprised 10 males and 4 females. Tumor locations included the femur (6 cases), tibia (3 cases), humerus (1 case), fibula (2 cases), ilium (1 case), and the posterior edge of the right deltoid muscle (1 case). The average tumor size was 3.6 ± 1.3&#xa0;cm. Most patients exhibited systemic osteomalacia, with 11 patients experiencing multiple rib fractures and an average preoperative disease duration of 29.04 ± 22.83 months. All patients demonstrated varying degrees of hypophosphatemia and elevated alkaline phosphatase levels, with PET/CT scans revealing increased FDG/DOTANOC uptake in the lesions. Postoperative follow-up indicated that patients who underwent tumor resection showed superior improvements in blood phosphorus levels and bone density compared to those who underwent curettage or ablation. The Musculoskeletal Tumor Society (MSTS) scores of all patients significantly improved postoperatively, increasing from a preoperative mean of 20.22 ± 3.44 to a postoperative mean of 28.38 ± 1.05 (<i>P</i> &lt; 0.05).</p> Conclusion <p>PMT is a rare and challenging mesenchymal tumor to diagnose due to its nonspecific symptoms and low incidence. The combination of DOTANOC-PET/CT imaging and postoperative pathological examination provides a reliable diagnostic approach. Among the surgical options, tumor resection has demonstrated superior efficacy in improving both biochemical markers and clinical outcomes, making it the preferred treatment method over tumor curettage or ablation.</p> Level of evidence <p>Level IV, therapeutic study.</p>

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Phosphaturic mesenchymal tumors: a report of 14 cases and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月21日

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  • Xirenijiang Yikemu,
  • Ajimu Keremu,
  • Changye Zou,
  • Yongqian Wang,
  • Wang Bo,
  • Zhiqiang Zhao,
  • Jian Tu,
  • Tiao Lin,
  • Junqiang Yin,
  • Jingnan Shen

摘要

Background

To review and study 14 cases of phosphaturic mesenchymal tumor (PMT) patients, integrating previous literature to expand understanding of this disease, improve diagnosis rates, and enhance treatment efficacy.

Methods

This study included 14 patients with PMT who were treated and pathologically confirmed at our bone tumor center between December 2016 and September 2023. Clinical data, including imaging, pathological findings, and laboratory results, were collected and analyzed. All patients underwent preoperative PET/CT scanning, followed by surgery, and were monitored during an average postoperative follow-up period of 28.86 ± 21.72 months. The surgical procedures included tumor resection, curettage, and ablation.

Results

The study cohort comprised 10 males and 4 females. Tumor locations included the femur (6 cases), tibia (3 cases), humerus (1 case), fibula (2 cases), ilium (1 case), and the posterior edge of the right deltoid muscle (1 case). The average tumor size was 3.6 ± 1.3 cm. Most patients exhibited systemic osteomalacia, with 11 patients experiencing multiple rib fractures and an average preoperative disease duration of 29.04 ± 22.83 months. All patients demonstrated varying degrees of hypophosphatemia and elevated alkaline phosphatase levels, with PET/CT scans revealing increased FDG/DOTANOC uptake in the lesions. Postoperative follow-up indicated that patients who underwent tumor resection showed superior improvements in blood phosphorus levels and bone density compared to those who underwent curettage or ablation. The Musculoskeletal Tumor Society (MSTS) scores of all patients significantly improved postoperatively, increasing from a preoperative mean of 20.22 ± 3.44 to a postoperative mean of 28.38 ± 1.05 (P < 0.05).

Conclusion

PMT is a rare and challenging mesenchymal tumor to diagnose due to its nonspecific symptoms and low incidence. The combination of DOTANOC-PET/CT imaging and postoperative pathological examination provides a reliable diagnostic approach. Among the surgical options, tumor resection has demonstrated superior efficacy in improving both biochemical markers and clinical outcomes, making it the preferred treatment method over tumor curettage or ablation.

Level of evidence

Level IV, therapeutic study.