Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia
A possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional...
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doaj-e2210d6fb2b541149b035b7999c7a96a2020-11-25T02:42:00ZengMDPI AGMedicina1010-660X2020-01-015613410.3390/medicina56010034medicina56010034Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic OsteomalaciaCornelia Then0Evelyn Asbach1Harald Bartsch2Niklas Thon3Christian Betz4Martin Reincke5Ralf Schmidmaier6Department of Endocrinology, Medizinische Klinik und Poliklinik IV, Ludwig-Maximilians-University, 80336 Munich, GermanyDepartment of Endocrinology, Medizinische Klinik und Poliklinik IV, Ludwig-Maximilians-University, 80336 Munich, GermanyInstitute of Pathology, Klinikum der Universität München, Ludwig-Maximilians-University, 80337 Munich, GermanyDepartment of Neurosurgery, Klinikum Universität München, Ludwig-Maximilians-University, 81377 Munich, GermanyDepartment of Otorhinolaryngology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyDepartment of Endocrinology, Medizinische Klinik und Poliklinik IV, Ludwig-Maximilians-University, 80336 Munich, GermanyDepartment of Endocrinology, Medizinische Klinik und Poliklinik IV, Ludwig-Maximilians-University, 80336 Munich, GermanyA possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional imaging. A 50-year-old woman presented with diffuse musculoskeletal pain and several fractures. Secondary causes of osteoporosis were excluded. Laboratory analysis revealed hypophosphatemia and elevated alkaline phosphatase, parathyroid hormone, and FGF-23. Thus, oncogenic osteomalacia due to neoplastic FGF-23 secretion was suspected. FDG-PET-CT and DOTATATE-PET-CT imaging demonstrated no tumor. Cranial MRI revealed a tumorous mass in the left cellulae ethmoidales. The tumor was resected and histopathological examination showed a cell-rich tumor with round to ovoid nuclei, sparse cytoplasm, and sparse matrix, resembling an olfactory neuroblastoma. Immunohistochemical analysis first led to diagnosis of olfactory neuroblastoma, which was later revised to phosphaturic mesenchymal tumor. Following the resection, FGF-23 and phosphate levels normalized. In conclusion, we here describe a patient with an FGF-23-secreting phosphaturic mesenchymal tumor with an unusual morphology. Furthermore, we emphasize diagnostic pitfalls when dealing with FGF-23-induced hypophosphatemia.https://www.mdpi.com/1010-660X/56/1/34fgf-23phosphaturic mesenchymal tumorpmthypophosphatemia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Cornelia Then Evelyn Asbach Harald Bartsch Niklas Thon Christian Betz Martin Reincke Ralf Schmidmaier |
spellingShingle |
Cornelia Then Evelyn Asbach Harald Bartsch Niklas Thon Christian Betz Martin Reincke Ralf Schmidmaier Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia Medicina fgf-23 phosphaturic mesenchymal tumor pmt hypophosphatemia |
author_facet |
Cornelia Then Evelyn Asbach Harald Bartsch Niklas Thon Christian Betz Martin Reincke Ralf Schmidmaier |
author_sort |
Cornelia Then |
title |
Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia |
title_short |
Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia |
title_full |
Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia |
title_fullStr |
Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia |
title_full_unstemmed |
Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia |
title_sort |
fibroblast growth factor 23-producing phosphaturic mesenchymal tumor with extraordinary morphology causing oncogenic osteomalacia |
publisher |
MDPI AG |
series |
Medicina |
issn |
1010-660X |
publishDate |
2020-01-01 |
description |
A possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional imaging. A 50-year-old woman presented with diffuse musculoskeletal pain and several fractures. Secondary causes of osteoporosis were excluded. Laboratory analysis revealed hypophosphatemia and elevated alkaline phosphatase, parathyroid hormone, and FGF-23. Thus, oncogenic osteomalacia due to neoplastic FGF-23 secretion was suspected. FDG-PET-CT and DOTATATE-PET-CT imaging demonstrated no tumor. Cranial MRI revealed a tumorous mass in the left cellulae ethmoidales. The tumor was resected and histopathological examination showed a cell-rich tumor with round to ovoid nuclei, sparse cytoplasm, and sparse matrix, resembling an olfactory neuroblastoma. Immunohistochemical analysis first led to diagnosis of olfactory neuroblastoma, which was later revised to phosphaturic mesenchymal tumor. Following the resection, FGF-23 and phosphate levels normalized. In conclusion, we here describe a patient with an FGF-23-secreting phosphaturic mesenchymal tumor with an unusual morphology. Furthermore, we emphasize diagnostic pitfalls when dealing with FGF-23-induced hypophosphatemia. |
topic |
fgf-23 phosphaturic mesenchymal tumor pmt hypophosphatemia |
url |
https://www.mdpi.com/1010-660X/56/1/34 |
work_keys_str_mv |
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