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Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor

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 t...

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Autores principales: Häfliger, Simon, Seidel, Ann-Katrin, Schoch, Eric, Reichmann, Jan, Wild, Damian, Steinmann-Schwager, Stephanie, Pless, Miklos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772845/
https://www.ncbi.nlm.nih.gov/pubmed/33442358
http://dx.doi.org/10.1159/000510334
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author Häfliger, Simon
Seidel, Ann-Katrin
Schoch, Eric
Reichmann, Jan
Wild, Damian
Steinmann-Schwager, Stephanie
Pless, Miklos
author_facet Häfliger, Simon
Seidel, Ann-Katrin
Schoch, Eric
Reichmann, Jan
Wild, Damian
Steinmann-Schwager, Stephanie
Pless, Miklos
author_sort Häfliger, Simon
collection PubMed
description 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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spelling pubmed-77728452021-01-12 Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor Häfliger, Simon Seidel, Ann-Katrin Schoch, Eric Reichmann, Jan Wild, Damian Steinmann-Schwager, Stephanie Pless, Miklos Case Rep Oncol Case Report 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. S. Karger AG 2020-11-30 /pmc/articles/PMC7772845/ /pubmed/33442358 http://dx.doi.org/10.1159/000510334 Text en Copyright © 2020 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Häfliger, Simon
Seidel, Ann-Katrin
Schoch, Eric
Reichmann, Jan
Wild, Damian
Steinmann-Schwager, Stephanie
Pless, Miklos
Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title_full Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title_fullStr Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title_full_unstemmed Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title_short Peptide Receptor Radionuclide Therapy for a Phosphaturic Mesenchymal Tumor
title_sort peptide receptor radionuclide therapy for a phosphaturic mesenchymal tumor
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772845/
https://www.ncbi.nlm.nih.gov/pubmed/33442358
http://dx.doi.org/10.1159/000510334
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