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Definition: Optic nerve meningioma (ONM) is a rare, benign tumor arising from arachnoid cap cells in meninges covering the optic nerve (ON).
Synonym: Optic nerve sheath meningioma (ONSM).
Classic Clue: Appears as an enhancing mass surrounding the nonenhancing ON with “tram track” calcifications and perioptic cysts.
The second most common ON tumor.
Calcification is a characteristic feature on computed tomography (CT).
Main differential diagnosis is optic nerve glioma (ONG) distinguishable by the absence of calcification.
High-density mass surrounding the ON.
Calcification in 20% to 50% of cases.
Postcontrast enhancement demonstrates the “tram track” or “sandwich” sign with the ON sandwiched between tumor masses.
Calcified ONMs may show the above appearance even without contrast.
Bony changes may include erosion and hyperostosis of the sphenoid and/or optic canal enlargement.
Computed tomography (CT) recommendations:
CT is less preferred but may be the first imaging study performed.
Radiation to the orbit is undesirable.
Less effective in demonstrating extent of disease.
Better at detecting calcifications. When present, calcifications are characteristically linear or punctate.
ONM may be isodense on non-enhancing (NE) CT depending on the amount of calcification.
“Tram tracking,” which may result from calcification or tumor enhancement
Calcification typically spares the distal ON and the optic disk.
Classic imaging appearance:
Isointense/or slightly hypointense (to brain) on T1-weighted images.
On T2-weighted images tumor signal varies inversely to the degree of calcification, from slightly hyperintense to hypointense.
More calcification produces a lower T2- signal.
Cerebrospinal fluid (CSF) in the dilated subarachnoid space gives characteristic perioptic cysts, best demonstrated on T2 weighted images (or by inversion recovery).
T1 Gd with mild enhancement is better demonstrated with fat saturation.
“Tram track” perineural enhancement is classic, but not pathognomonic. See Differential Diagnosis section.
The intracranial component of this tumor may enhance more than the intraorbital component.
Contrast enhanced magnetic resonance imaging (MRI) is:
The preferred imaging procedure.
MRI with Gd and fat saturation can detect and demarcate ONM with a precision not attainable with any other current imaging technique.
Better at defining disease extent, which may involve orbital apex, optic canal, and intracranial structures.
Better at excluding intracranial origin of en plaque meningioma from planum sphenoidale and tuberculum sellae.
It is noteworthy that 90% of optic nerve gliomas (ONGs) originate outside the orbit.
Better at demonstrating other findings in patients who have or may have neurofibromatosis (NF).
Allows sequential follow-up of patient without increasing cumulative radiation dose.
Fat saturation imaging is helpful in evaluating subtle lesions.
Rare.
Presentation: Progressive vision loss or proptosis.
Usually unilateral.
Two thirds occur in middle aged females.
Mean onset age 45 to 49 years (depending on series).
Suggests neurofibromatosis type 2 (NF2).
NF2 is a risk factor for ONM.
9% of patients with ONM have NF2.
Approximately 10,000 meningiomas diagnosed in the United States annually.
Approximately 100 ONMs diagnosed in the United States annually.
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