Juvenile psammomatoid ossifying fibroma of the neurocranium

Abstract

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.

Psammomatoid Juvenile Ossifying Fibroma of Maxilla With Secondary Aneurysmal Bone Cyst: A Case Report With Review of Literature.

“…(7) Aggressive growth occurs in some but not all cases encroaching adjacent orbital, nasal, and cranial compartments, distorting the face, displacing orbital contents, and blocking normal sinus drainage to form mucoceles. Intracranial extension develops slowly with few neurological signs or symptoms, even though the lesions may be large enough to fill the anterior cranial fossa (12). The dura maintains an effective barrier to invasion of brain.…”

“…The radiographic appearance manifests as well-demarcated, unilocular or less commonly multilocular radiolucencies with a variable amount of radiopacity, usually manifesting as fine flakes or as ground-glass opacification (5)(6)(7)(8)(9)(10)(11)(12)(13)(14). Aggressive forms tend to show more expansile growth pattern than the typical variety.…”

Cranial juvenile psammomatoid ossifying fibroma: case report

“…8 The proliferation index (Ki 67) is frequently low and not useful to predict aggressive behavior. 10 Little is known about the aggressive biological behavior of this lesion.…”

“…16 The etiology and the predictive factors of cystic transformation remain unclear, but they may be related to genetic and/or environmental causes. 10 Clinically cyst formation has been the cause of accelerated tumor growth in a short period of time, although patients remain pain free and with no neurological deficit. The cyst occurs more commonly in children.…”

“…These lesions produce "cementum-like" material, structures called "droplets" or "cementicles," which show oval mineralized spherules. 10 The clinical differential diagnosis includes primary extracranial psammomatoid meningioma (PEPM). Juvenile psammomatoid ossifying fibroma may be difficult to distinguish from PEPM given the macroscopic anatomical superficial similarity, neurocranial location, and radiological aspects.…”