Abstract
BackgroundTo evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy.MethodsPatient data from patients admitted to our institution during a 217-month period from August 1999 to September 2017 were included. Patient ophthalmic manifestations, radiographic features (CT and MRI), diagnosis, pathology, therapeutic regimens, and prognosis were retrospectively reviewed.ResultsSix patients with ectopic orbital meningioma were identified. The mean age at the first visit was 33.2 years (range, 7–56 years). All six patients displayed manifestations of exophthalmos, upper eyelid oedema, and motility impairment with a mean history of illness of 20.3 months (range 3–72 months). Optical lesions were located in the superonasal extraconal compartment (3/6, 50%), bitemporal extraconal compartment (1/6, 16.7%) and orbital intraconal compartment (2/6, 33%). Radiographic features were ill-defined, heterogeneous, enhancing soft tissue masses with extraocular muscular adhesion (6/6, 100%) and calcification (1/6, 16.7%), not adjacent to the optic nerve and not extending along the dura. Six cases were treated intraoperatively with complete surgical resection, indicating that all lesions were independent of the optic nerve and sphenoid ridge. The histopathologic classification was mostly of meningothelial cells (5/6, 83%). Immunohistochemistry revealed EMA and vimentin to have positive expression in all six cases, while two cases were calponin-positive and strongly expressed in the olfactory bulb. Postoperatively, lesions caused no visual impairment, and there were no cases of recurrence.ConclusionsEctopic orbital meningiomas are rare tumours that are not easily diagnosed without postoperative histopathology. This report highlights some of the distinguishing features of isolated orbital lesions, especially around the location of frontoethmoidal suture. Accompanying upper eyelid oedema and eye mobility restriction were observed to be dissimilar to other orbital tumours. In these cases, a diagnosis of ectopic orbital meningioma should be considered.
Highlights
To evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy
Primary orbital meningioma accounted for 5–10% of all orbital tumours and 30% of all orbital meningiomas; they were mainly observed in adults and rarely in children [3]
The main complaints recorded at the first visit were upper eyelid oedema (6/6, 100%), exophthalmos (5/6, 83%), ptosis (4/6, 66.7%), impaired vision (2/6, 33%), diplopia (2/6, 33%) and tumours detected by physical examination (2/6, 33%)
Summary
To evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy. Meningiomas are a variety of tumours caused by arachnoid “cap” cells of meningeal arachnoid villi [1]. Orbital meningiomas can be considered to be primary and secondary in origin [2]. Primary orbital meningioma accounted for 5–10% of all orbital tumours and 30% of all orbital meningiomas; they were mainly observed in adults and rarely in children [3]. A rare subset of orbital meningiomas that do not involve the optic nerve sheath or sphenoid ridge were initially considered to be “ectopic”. Ectopic orbital meningiomas are occasionally reported as single or multiple case series in the literature. There exists a paucity of published clinical evidence regarding the distinguishing features of ectopic orbital meningioma.
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