Oncogenic osteomalacia secondary to phosphaturic mesenchymal tumor
Updates to Case Attributes
Oncogenic osteomalacia or tumour induced osteomalacia, is an acquired para-neoplastic syndrome
Cases typically are diagnosed in 6th decade of life
Tumours that can lead to this syndrome are classified histologically as:
- Phosphaturic mesenchymal tumours with mixed connective tissue (70 -80%)
- Osteoblastoma like tumours
- Ossifying fibrous like tumours
- Nonossifying fibrous like tumours
These tumours are often small in size and difficult to localize
The average time between onset of symptoms and tumour localization is ~ 7 years
These tumours often express somatostatin receptors
Therefore, scintigraphy using somatostatin analogue can be used for tumour localization
Cases can have elevated serum Fibroblast Growth Factor 23 (FGF-23) levels
FDG PET scan can be used for tumour localisation and is been increasingly used
Treatment:
Tumour resection is the preferred treatment option
Post-surgery FGF-23 levels fall off drastically
Serum phosphate and 1,25-dihydroxy vitamin D levels return to normal levels within days
Long term skeletal changes reverse within months
-</ol><p> </p><p>These tumours are often <strong>small in size and difficult to localize</strong></p><p>The average time between onset of symptoms and tumour localization ~ 7 years</p><p>These tumours often express somatostatin receptors</p><p>Therefore, scintigraphy using somatostatin analogue can be used for tumour localization</p><p>Cases can have elevated serum Fibroblast Growth Factor 23 (FGF-23) levels</p><p>FDG PET scan can be used for tumour localisation and is been increasingly used</p><p> </p><p>Treatment:</p><p>Tumour resection is the preferred treatment option</p><p>Post-surgery FGF-23 levels fall off drastically</p><p>Serum phosphate and 1,25-dihydroxy vitamin D levels return to normal levels within days</p><p>Long term skeletal changes reverse within months</p><p> </p><p> </p><p> </p>- +</ol><p> </p><p>These tumours are often <strong>small in size and difficult to localize</strong></p><p>The average time between onset of symptoms and tumour localization is ~ 7 years</p><p>These tumours often express somatostatin receptors</p><p>Therefore, scintigraphy using somatostatin analogue can be used for tumour localization</p><p>Cases can have elevated serum Fibroblast Growth Factor 23 (FGF-23) levels</p><p>FDG PET scan can be used for tumour localisation and is been increasingly used</p><p> </p><p>Treatment:</p><p>Tumour resection is the preferred treatment option</p><p>Post-surgery FGF-23 levels fall off drastically</p><p>Serum phosphate and 1,25-dihydroxy vitamin D levels return to normal levels within days</p><p>Long term skeletal changes reverse within months</p><p> </p><p> </p><p> </p>
References changed:
- 1. Weidner N. Review and Update: Oncogenic Osteomalacia-Rickets. Ultrastruct Pathol. 1991;15(4-5):317-33. <a href="https://doi.org/10.3109/01913129109016242">doi:10.3109/01913129109016242</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1755097">Pubmed</a>
- 3. Folpe A, Fanburg-Smith J, Billings S et al. Most Osteomalacia-Associated Mesenchymal Tumors Are a Single Histopathologic Entity. Am J Surg Pathol. 2004;28(1):1-30. <a href="https://doi.org/10.1097/00000478-200401000-00001">doi:10.1097/00000478-200401000-00001</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/14707860">Pubmed</a>
- 2. Chong W, Molinolo A, Chen C, Collins M. Tumor-Induced Osteomalacia. Endocr Relat Cancer. 2011;18(3):R53-77. <a href="https://doi.org/10.1530/erc-11-0006">doi:10.1530/erc-11-0006</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/21490240">Pubmed</a>
Updates to Study Attributes
PoolingCompared to the previous 'blood pool scintigraphy' done 8 years ago, the present study demonstrated increased tracer pooling the region of tracer in the upper nasal and left frontal regionanterior skull base.
MRI scan was done for further evaluation.
Updates to Study Attributes
Well defined lobulated solid cystic mass in the left basifrontal region with extension into the olfactory groove. Surrounding mass effect with midline shift to right.
Image MRI (T2 fat sat) ( update )
Updates to Study Attributes
Blood pool scintigraphy with pooling of tracer in the region of right upper nasal cavityanterior skull base.
Updates to Study Attributes
CT scan did not demonstrate any obvious abnormality in the upper nasalanterior skull base region.
Updates to Freetext Attributes
Summary till now:
Middle
-
Middle aged woman
Progressive -
Progressive bone pains with difficulty in walking
On -
On imaging severe osteopenia with multiple fractures
On - On blood investigations hypophosphatemia
Provisional diagnosis:
- Adult onset hypophosphatemic osteomalacia.
UndeterminedEvaluated to assess the cause.