Expectations of Response from Octreotide Therapy in Recurrent Phosphaturic Mesenchymal Tumors - Do They Reflect Reality?

Ulaş, Arife; Dede, Didem Şener; Şendur, Mehmet Ali Nahit; Akıncı, Muhammed Bülent; Yalçın, Bülent

doi:10.7314/apjcp.2014.15.24.10997

Cited by 3 publications

(3 citation statements)

References 14 publications

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“…The PMT-MCT frequently has an infiltrating growth pattern, as reported in the present cases, and this may explain the high rates of local recurrence ( 44 , 60 – 64 ). Early, complete surgical resection remains the definitive treatment of PMT-MCTs.…”

Section: Discussionsupporting

confidence: 72%

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CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report

Shi

Deng²,

et al. 2018

Oncol Lett

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Phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT) is rare and usually benign and slow-growing. The majority of these tumors is associated with sporadic tumor-induced osteomalacia (TIO) or rickets, affect middle-aged individuals and are located in the extremities. Previous imaging studies often focused on seeking the causative tumors of TIO, not on the radiological features of these tumors, especially magnetic resonance imaging (MRI) features. PMT-MCT remains a largely misdiagnosed, ignored or unknown entity by most radiologists and clinicians. In the present case report, a review of the known literature of PMT-MCT was conducted and the CT and MRI findings from three patient cases were described for diagnosing the small subcutaneous tumor. Typical MRI appearances of PMT-MCT were isointense relative to the muscles on T1-weighted imaging, and markedly hyperintense on T2-weighted imaging containing variably flow voids, with markedly heterogeneous/homogenous enhancement on post contrast T1-weighted fat-suppression imaging. Short time inversion recovery was demonstrated to be the optimal sequence in localizing the tumor.

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Section: Discussionsupporting

confidence: 72%

“…Although somatostatin receptors have been identified on PMT-MCTs, octreotide therapy, which was based on decreasing FGF23 secretion, has not been effective. Periodic measurement of serum phosphate and FGF23 levels has been advocated as a means to detect recurrences ( 3 , 60 , 68 ).…”

Section: Discussionmentioning

confidence: 99%

CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report

Shi

Deng²,

et al. 2018

Oncol Lett

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“…Neither of these cases showed a clinical or biochemical response. Thus, the current value of octreotide for the treatment of PMT remains unclear [10, 11].…”

Section: Introductionmentioning

confidence: 99%

Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor

et al. 2020

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Tumor-induced osteomalacia is a very rare paraneoplastic syndrome. It can be caused by phosphaturic mesenchymal tumor (PMT), a generally benign tumor that produces fibroblast growth factor 23 (FGF-23), which can cause a severe renal phosphate wasting syndrome. Upon complete surgical removal of the tumor, FGF-23 normalizes and the osteomalacia is cured. In cases in which surgery is not feasible, radiofrequency ablation (RFA) is the treatment of choice. We describe a case with a PMT situated in the sacrum, in close proximity to the sacral plexus. Both surgery and RFA were considered potentially nerve damaging. Since the tumor showed expression of somatostatin receptors, we opted for a peptide receptor radionuclide therapy (PRRT) with <sup>177</sup>Lu-DOTATOC. However, the therapy did not show the expected success, since the FGF-23 level had even temporarily increased. The patient was then successfully treated with RFA. A partial remission of the tumor was achieved and FGF-23 levels nearly normalized. Despite some severe neurological side effects, the patient showed a remarkable clinical improvement, with no symptoms of osteomalacia within a few weeks.

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Phosphaturic mesenchymal tumor: two cases highlighting differences in clinical and radiologic presentation

Gu,

Ge,

Joshi

et al. 2023

Skeletal Radiol

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Expectations of Response from Octreotide Therapy in Recurrent Phosphaturic Mesenchymal Tumors - Do They Reflect Reality?

Cited by 3 publications

References 14 publications

CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report

CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report

Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor

Phosphaturic mesenchymal tumor: two cases highlighting differences in clinical and radiologic presentation

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