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. 2022 Dec 29;12(1):252.
doi: 10.3390/jcm12010252.

Phosphaturic Mesenchymal Tumors: Rethinking the Clinical Diagnosis and Surgical Treatment

Yupeng Liu  1   2 , Hongbo He  1   2 , Can Zhang  1   2 , Hao Zeng  1 , Xiaopeng Tong  1 , Qing Liu  1   2
Affiliations

Phosphaturic Mesenchymal Tumors: Rethinking the Clinical Diagnosis and Surgical Treatment

Yupeng Liu et al. J Clin Med. .

Abstract

Background: The diagnosis of phosphaturic mesenchymal tumors (PMT) is easily delayed clinically, and their surgical treatment is unstandardized. This study aimed to evaluate our experience in the diagnosis and treatment of PMT and provide a research basis for the accurate and standardized treatment of PMT.

Materials and methods: Twelve patients diagnosed with PMT in our department and who underwent surgical treatment were included in this study. Preoperative demographic and clinical information were recorded. CT, MRI, and technetium-99m (Tc99m)-octreotide PET/CT imaging techniques were used to evaluate the general conditions and lesion boundaries of the tumors. Surgical treatment was performed using radical resection and microwave ablation-assisted extended curettage according to the lesion location and size. Patients were strictly followed up with and evaluated for oncological prognosis, radiological results, bone healing, serum ion levels, limb function, and pain level; the occurrence of complications was also recorded.

Results: Three patients underwent radical resection, and nine underwent microwave ablation-assisted extended curettage. The average duration of symptoms in this group was 1.5 years (9-35 months) before diagnosis. Serum phosphate and AKP levels returned to normal one and two weeks postoperatively, respectively. There was no apparent specificity in the pathological findings; however, the immunohistochemistry of FGF-23 was positive, and the original fracture sites were effectively healed during the follow-up. The limb function and pain scores were significantly improved. The MSTS score increased from 15.3 to 29.0, and the VAS score decreased from 5.3 to 0.4. All patients recovered, and 90% resumed their original jobs.

Conclusions: Accurate diagnosis and standardized surgical treatment are crucial to achieving a clinical cure for PMT. Combining clinical manifestations, biochemical examinations, imaging characteristics, and pathological findings is an effective way to diagnose PMT accurately. Radical resection and microwave ablation-assisted extended curettage are reliable surgical treatment methods for PMT.

Keywords: FGF-23; Tc99m-octreotide PET/CT; hypophosphataemia; phosphaturic mesenchymal tumors; tumor-induced osteomalacia.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Figure 1
Figure 1
Case 4: PMT of the proximal femur that underwent radical resection and tu-mor-type artificial joint replacement. (a) Anteroposterior and lateral X-rays of the proximal femur revealed intertrochanteric bone abnormalities. (b) MRI showed low intensity on T1WI and high intensity on T2WI (white arrow indicates the lesion). (c) CT showed heterogeneous lesion density with osteogenic changes (white arrow indicates the lesion). (d) Tumor segment resection and tumor-type artificial hip replacement were performed. (e) Postoperative X-ray showed that the prosthesis was stable and the lower limbs were basically equal in length.
Figure 2
Figure 2
Case 10: PMT of the right proximal femur with pathological fracture; microwave ablation-assisted extended curettage was performed, and bone graft filling was assisted by an in-ternal fixation device. (a) Anteroposterior X-ray of the proximal right femur (white arrow indicates the lesion). (b) CT scan revealed an osteolytic lesion with a marked sclerosing margin below the lesser trochanter of the right femur (white arrow indicates the lesion). (c) MRI showed low intensity on T1WI and high intensity on T2WI, with obvious en-hancement after lipid suppression enhancement. (d) Tc99m-octreotide PET/CT scan accurately located the lesions and showed abnormal concentration. (e) Intraoperative details: microwave ablation was used to inactivate the lesions at the beginning. (f) Postoperative X-rays of the an-teroposterior and lateral after extensive curettage of the lesion with bone graft filling and proximal femoral anatomical plate fixation.
Figure 3
Figure 3
The results of the pathological examination of PMT showed that the staining was mostly spindle cells and bone-like matrix components, and the immunohistochemical staining of FGF23, SSTR2A, and SATB2 was strongly positive.
Figure 4
Figure 4
Case 5: PMT of the middle tibia with pathologic fracture underwent microwave ablation-assisted extended curettage. (a) Anteroposterior and lateral X-rays showed osteolytic lesions in the middle tibia with pathological fractures and obvious hardening of the fracture ends. (b) Tc99m-octreotide PET/CT scan showed an abnormal concentration of the lesion. (c,d) MRI showed low intensity on T1WI and high intensity on T2WI, with obvious enhancement after lipid suppression enhancement. (e) Microwave ablation-assisted extended curettage was performed, followed by cement filling and assisted by an internal fixation device.
Figure 5
Figure 5
Case 2: PMT of the left iliac crest was treated with radical resection and cement filling. (a) Anteroposterior and oblique X-rays of the pelvis. (b) Tc99m-octreotide PET/CT scan accurately located the lesions and showed abnormal concentration. (c) The 3D printing technology reproduces the lesion and designs the osteotomy guide plate. (d) Postoperative anteroposterior X-ray of the pelvis.
Figure 6
Figure 6
Flow chart of accurate diagnosis of phosphaturic mesenchymal tumors.
See this image and copyright information in PMC

References

    1. Hartley I.R., Roszko K.L., Li X., Pozo K., Streit J., Del R.J., Magone M.T., Smith M.R., Vold R., Dambkowski C.L., et al. Infigratinib reduces fibroblast growth factor 23 (fgf23) and increases blood phosphate in tumor-induced osteomalacia. JBMR Plus. 2022;6:e10661. doi: 10.1002/jbm4.10661. - DOI - PMC - PubMed
    1. Hidaka N., Koga M., Kimura S., Hoshino Y., Kato H., Kinoshita Y., Makita N., Nangaku M., Horiguchi K., Furukawa Y., et al. Clinical challenges in diagnosis, tumor localization and treatment of tumor-induced osteomalacia: Outcome of a retrospective surveillance. J. Bone Miner. Res. 2022;37:1479–1488. doi: 10.1002/jbmr.4620. - DOI - PubMed
    1. Rendina D., Abate V., Cacace G., D’Elia L., De Filippo G., Del V.S., Galletti F., Cuocolo A., Strazzullo P. Tumor-induced osteomalacia: A systematic review and individual patient’s data analysis. J. Clin. Endocrinol. Metab. 2022;107:e3428–e3436. doi: 10.1210/clinem/dgac253. - DOI - PubMed
    1. Cianferotti L., Delli P.C., Bertoldo F., Caffarelli C., Crotti C., Gatti D., Giannini S., Gonnelli S., Mazzantini M., Ombretta V., et al. Persistence and recurrence in tumor-induced osteomalacia: A systematic review of the literature and results from a national survey/case series. Endocrine. 2022;76:709–721. doi: 10.1007/s12020-022-03039-2. - DOI - PMC - PubMed
    1. Benson J.C., Trejo-Lopez J.A., Nassiri A.M., Eschbacher K., Link M.J., Driscoll C.L., Tiegs R.D., Sfeir J., Delone D.R. Phosphaturic mesenchymal tumor. Am. J. Neuroradiol. 2022;43:817–822. doi: 10.3174/ajnr.A7513. - DOI - PMC - PubMed

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