Phosphaturic Mesenchymal Tumor

Barrera, Christian A.; Karp, John; Kearns, Ciléin; Rhodes, Nicholas G.

doi:10.1148/rg.220185

Cited by 3 publications

(7 citation statements)

References 6 publications

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“…The calcified matrix components with osteoclast‐like giant cells, and metaplastic bone tissue, exhibit characteristics similar to cartilage or bone tissue 12 . The marked biochemical characteristics include hypophosphatemia caused by renal phosphate consumption, a near normal or significant decrease in 1,25‐dihydroxy vitamin D, and a near normal or elevated level of FGF23 13,14 …”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Most PMTs can be completely cured through tumor resection surgery, and hypophosphatemia and osteomalacia can alleviate subsequently 9 . In cases where the tumor cannot be localized or completely resected, image guided ablation combined with cryoablation or radiofrequency ablation can be used as an alternative treatment 9,13 . Small molecule targeted drugs can significantly improve symptoms and inhibit disease progression in unresectable, relatively concealed, or unidentified PMTs, but there is still a lack of evidence regarding their long‐term efficacy and safety 14,15 .…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“… 12 The marked biochemical characteristics include hypophosphatemia caused by renal phosphate consumption, a near normal or significant decrease in 1,25‐dihydroxy vitamin D, and a near normal or elevated level of FGF23. 13 , 14 …”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…12 The marked biochemical characteristics include hypophosphatemia caused by renal phosphate consumption, a near normal or significant decrease in 1,25-dihydroxy vitamin D, and a near normal or elevated level of FGF23. 13,14 Medical treatment is generally performed with phosphate plus active vitamin D (calcitriol or alpha calcitriol). Most PMTs can be completely cured through tumor resection surgery, and hypophosphatemia and osteomalacia can alleviate subsequently.…”

Section: Literature Reviewmentioning

confidence: 99%

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Tumor‐induced Osteomalacia: A Case Report and Etiological Analysis with Literature Review

Zhang,

Li,

Zhang

et al. 2023

Orthopaedic Surgery

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BackgroundTumor‐induced osteomalacia (TIO) belongs to a rare disease of the paraneoplastic syndrome. Phosphate uric mesenchymal tumor (PMT) is the most common cause of TIO, while the possibility of other tumors cannot be excluded.Case presentationWe present a case of a 36‐year‐old female patient with systemic skeletal abnormalities. The woman complained of low back pain with mild motor dysfunction for 2 years. Laboratory examination showed abnormalities in markers of bone metabolism, parathyroid hormone (PTH), vitamin D and serum phosphorus. Pooled imaging examination indicated extension abnormalities in the skeletal system and a single lesion in the right femoral head. The lesion of the right femoral was imaging with somatostatin receptor‐positive, which was highly suggestive of a single neuroendocrine tumor. CT guided right femoral tumorectomy and bone grafting were performed when medical treatment failed. Postoperative pathological diagnosis was phosphate urinary mesenchymal tumor secreting fibroblast growth factor 23 (FGF23), which accorded with pre‐operative expectations. The postoperative symptoms were effectively relieved, and indicators returned to normal.ConclusionThe tumors causing TIO exhibited significant heterogeneity in terms of tissue origin, pathological characteristics and biological behavior, but the unique common characteristic is the secretion of FGF23. With significant progress in diagnosis and treatment, the clinical follow‐up of most TIO patients shows a good prognosis, but the prognosis of those with malignant tumors is relatively poor.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

See 2 more Smart Citations

Tumor‐induced Osteomalacia: A Case Report and Etiological Analysis with Literature Review

Zhang,

Li,

Zhang

et al. 2023

Orthopaedic Surgery

View full text Add to dashboard Cite

show abstract

“…5 The typical laboratory examination manifestations of PMTs include hypophosphatemia and relative hyperphosphaturia, patients may also have biochemical changes such as an increase in ALP, and low to low-normal serum calcium, 1,25 dihydroxyvitamin D and parathormone levels. 6 Notably, patients with PMTs often have a high serum FGF-23 levels. Selective venous blood sampling for the detection of the FGF-23 concentration can assist in tumor localization, but FGF-23 detection is not currently widely applied in clinical practice.…”

Section: F I G U R Ementioning

confidence: 99%

Challenging diagnosis and successful management of phosphaturic mesenchymal tumor mimicking ankylosing spondylitis: A case report

Zhang,

Zhu,

Chen

et al. 2024

Int J of Rheum Dis

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Phosphaturic Mesenchymal Tumor-induced Osteomalacia

2023

J Surg

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Tumor Induced Osteomalacia (TIO) is a rare paraneoplastic disorder characterized by hypophosphatemia, high serum alkaline phosphatase, reduced active vitamin D, suboptimal bone mineral density, bone pain, fragility fractures, and muscle weakness. This paraneoplastic disorder is caused by secretion of Fibroblast Growth Factor 23 (FGF23). In TIO, FGF23 is secreted by mesenchymal tumors that are usually benign, but are typically very small and therefore difficult to locate. Diagnosis of this disease is often challenging. In this article we describe two clinical cases, diagnosed and treated at our institution. The two patients suffered from bone pain, severe osteoporosis and hypophosphatemia for several years. Ga68-DOTANOC PET/CT was very effective in localizing the tumors. In both cases, complete tumor resection was performed, resulting in immediate normalization of FGF23, phosphorus and vitamin D. Subsequent microscopic examination revealed a phosphaturic mesenchymal tumor. The authors emphasize the importance of the diagnosis of this disease that leads to surgical removal of the causative tumor and consequently the prevention of severe disability and perhaps even death.

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Phosphaturic Mesenchymal Tumor

Cited by 3 publications

References 6 publications

Tumor‐induced Osteomalacia: A Case Report and Etiological Analysis with Literature Review

Tumor‐induced Osteomalacia: A Case Report and Etiological Analysis with Literature Review

Challenging diagnosis and successful management of phosphaturic mesenchymal tumor mimicking ankylosing spondylitis: A case report

Phosphaturic Mesenchymal Tumor-induced Osteomalacia

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