Phosphaturic mesenchymal tumor : management and outcomes of ten patients treated at a single institution
© 2024. The Author(s), under exclusive licence to International Skeletal Society (ISS)..
BACKGROUND: Phosphaturic mesenchymal tumor (PMT) is a rare tumor that causes tumor-induced osteomalacia. Patients present with non-specific symptoms secondary to renal phosphate wasting and decreased bone mineralization. We sought to assess: (1) What are the common presenting features, laboratory and imaging findings, histologic findings of phosphaturic mesenchymal tumors? (2) What are the available treatment strategies for phosphaturic mesenchymal tumors and their long-term outcomes in terms of local recurrence and symptom control after treatment?.
METHODS: We retrospectively identified patients with a histologic diagnosis of PMT located in the axial or appendicular skeleton, or surrounding soft tissues. A total of 10 patients were finally included in our study.
RESULTS: Median tumor size was 1.9 cm (range, 1.1 to 6.1) and median time from symptom onset to diagnosis was 3 years (range, 0.5 to 15 years). All patients but one presented with hypophosphatemia (median 1.9 mg/dL, range 1.2 to 3.2). Pre-operative FGF-23 was elevated in all cases (median 423.5 RU/mL, range 235 to 8950). Six patients underwent surgical resection, three were treated percutaneously (radiofrequency ablation or cryoablation), and one refused treatment. Only one patient developed local recurrence and no patients developed metastatic disease. At last follow-up, nine patients showed no evidence of disease and one was alive with disease.
CONCLUSION: Phosphaturic mesenchymal tumor is a rare tumor presenting with non-specific symptoms. Surgery is the standard treatment when negative margins can be achieved without significant morbidity. In patients with small tumors in surgically-inaccessible areas, radiofrequency ablation or cryoablation can be performed successfully.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Skeletal radiology - (2024) vom: 13. Feb. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Gonzalez, Marcos R [VerfasserIn] |
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| Links: |
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| Themen: |
Cryoablation |
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| Anmerkungen: |
Date Revised 14.02.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1007/s00256-024-04614-6 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM368415155 |
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| 245 | 1 | 0 | |a Phosphaturic mesenchymal tumor |b management and outcomes of ten patients treated at a single institution |
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| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status Publisher | ||
| 520 | |a © 2024. The Author(s), under exclusive licence to International Skeletal Society (ISS). | ||
| 520 | |a BACKGROUND: Phosphaturic mesenchymal tumor (PMT) is a rare tumor that causes tumor-induced osteomalacia. Patients present with non-specific symptoms secondary to renal phosphate wasting and decreased bone mineralization. We sought to assess: (1) What are the common presenting features, laboratory and imaging findings, histologic findings of phosphaturic mesenchymal tumors? (2) What are the available treatment strategies for phosphaturic mesenchymal tumors and their long-term outcomes in terms of local recurrence and symptom control after treatment? | ||
| 520 | |a METHODS: We retrospectively identified patients with a histologic diagnosis of PMT located in the axial or appendicular skeleton, or surrounding soft tissues. A total of 10 patients were finally included in our study | ||
| 520 | |a RESULTS: Median tumor size was 1.9 cm (range, 1.1 to 6.1) and median time from symptom onset to diagnosis was 3 years (range, 0.5 to 15 years). All patients but one presented with hypophosphatemia (median 1.9 mg/dL, range 1.2 to 3.2). Pre-operative FGF-23 was elevated in all cases (median 423.5 RU/mL, range 235 to 8950). Six patients underwent surgical resection, three were treated percutaneously (radiofrequency ablation or cryoablation), and one refused treatment. Only one patient developed local recurrence and no patients developed metastatic disease. At last follow-up, nine patients showed no evidence of disease and one was alive with disease | ||
| 520 | |a CONCLUSION: Phosphaturic mesenchymal tumor is a rare tumor presenting with non-specific symptoms. Surgery is the standard treatment when negative margins can be achieved without significant morbidity. In patients with small tumors in surgically-inaccessible areas, radiofrequency ablation or cryoablation can be performed successfully | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Cryoablation | |
| 650 | 4 | |a Phosphaturic mesenchymal tumor | |
| 650 | 4 | |a Radiofrequency ablation | |
| 650 | 4 | |a Surgery | |
| 700 | 1 | |a Patel, Neel |e verfasserin |4 aut | |
| 700 | 1 | |a Connolly, Joseph J |e verfasserin |4 aut | |
| 700 | 1 | |a Hung, Yin P |e verfasserin |4 aut | |
| 700 | 1 | |a Chang, Connie Y |e verfasserin |4 aut | |
| 700 | 1 | |a Lozano-Calderon, Santiago A |e verfasserin |4 aut | |
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