Juvenile psammomatoid ossifying fibroma of the neurocranium. Report of four cases.
Juvenile psammomatoid ossifying fibroma (JPOF) is a benign fibroosseous lesion predominantly arising within the paranasal sinuses in children and young adults. Neurocranial occurrence is exceedingly rare and a location within the neurocranial portion of the temporal bone has not been described. The authors report on one case of sinonasal JPOF secondarily extending into the cranial cavity and three cases primarily affecting the neurocranial bones to increase clinical awareness of this uncommon tumor, which may be easily mistaken for meningioma. Moreover, the absence of activating missense mutations of the GNAS1 gene in two cases strongly argues against a relationship between JPOF and fibrous dysplasia.
Hasselblatt
Martin
M
Institute of Neuropathology, University Hospital Münster, Germany. hasselblatt@uni-muenster.de
Jundt
Gernot
G
Greiner
Christoph
C
Rama
Burckhard
B
Schmäl
Frank
F
Iglesias-Rozas
José R
JR
van de Nes
Johannes A P
JA
Paulus
Werner
W
D000293:Adolescent; D000328:Adult; D002864:Chromogranins; D003937:Diagnosis, Differential; D005260:Female; D018214:Fibroma, Ossifying / Q000473:pathology*; D005357:Fibrous Dysplasia of Bone / Q000235:genetics / Q000473:pathology; D019205:GTP-Binding Protein alpha Subunits, Gs / Q000235:genetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000473:pathology; D008579:Meningioma / Q000473:pathology; D010255:Paranasal Sinus Neoplasms / Q000473:pathology*; D012888:Skull Neoplasms / Q000473:pathology*; D013701:Temporal Bone / Q000473:pathology*
D002864:Chromogranins; C406732:GNAS protein, human; D019205:GTP-Binding Protein alpha Subunits, Gs