Intracranial phosphaturic mesenchymal tumors: report of 2 cases.
Hypophosphatemia with osteomalacia may be due to a neoplasm that produces fibroblast growth factor 23 (FGF-23), which inhibits phosphate reabsorption in the kidneys. Most of these tumors occur in bone or soft tissue and occasionally in the head, although intracranial occurrence is very rare. This report describes a tumor that caused hypophosphatemia and osteomalacia and was located entirely in the right anterior cranial fossa. Radiologically, the tumor resembled a meningioma; histologically, it was a low-grade phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT). After gross-total resection, the patient's symptoms abated and laboratory values normalized. The authors also studied another PMTMCT initially diagnosed as a hemangiopericytoma that involved the left anterior cranial fossa and ethmoid sinus, and reviewed reports of 6 other intracranial tumors that induced osteomalacia, 3 entirely in the anterior cranial fossa, 2 involving the anterior cranial fossa and ethmoid sinus, and 1 in the cavernous sinus. In older children or adults who have hypophosphatemia with osteomalacia and no personal or family history of metabolic, renal, or malabsorptive disease, a neoplasm should be suspected and an imaging workup that includes the brain is warranted, with particular attention to the anterior cranial fossa. Additionally, because there are some overlapping histological features between PMTMCTs and hemangiopericytomas, it may be helpful to assess tumoral FGF-23 expression by reverse transcriptase polymerase chain reaction or immunohistochemical analysis in patients with oncogenic osteomalacia from an intracranial tumor diagnosed as, or resembling, hemangiopericytoma.
Division of Neuropathology, The University of Texas Southwestern Medical Center, Dallas, TX, USA.
Derek A
DA
Mathis
Edward J
EJ
Stehel
Joseph E
JE
Beshay
Bruce E
BE
Mickey
Andrew L
AL
Folpe
Jack
J
Raisanen
D000328:Adult; D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D000089703:Fibroblast Growth Factor-23; D005346:Fibroblast Growth Factors / Q000378:metabolism; D015972:Gene Expression Regulation, Neoplastic; D006393:Hemangiopericytoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D017674:Hypophosphatemia / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D008637:Mesenchymoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D019635:Neurosurgical Procedures; D010018:Osteomalacia / Q000175:diagnosis* / Q000209:etiology; D016896:Treatment Outcome
C000717427:FGF23 protein, human; D005346:Fibroblast Growth Factors; D000089703:Fibroblast Growth Factor-23