A primary optic nerve sheath chordoid meningioma

Cases Journal, 2008

A case of a 75-year old male with right-sided exopthalmos is presented. Outside proptosis of the right eye was initially observed 6 years ago. Opthalmological and endocrinological clinical examination as well as laboratory tests revealed no pathology from optic nerve disc, optic bulb and thyroid related hormones. MRI study demonstrated an optic nerve meningioma. The key imaging findings and the differential diagnosis were discussed in this present paper.

Seminars in Ophthalmology, 2013

Meningiomas are benign neoplastic lesions of arachnoidal cells of the meninges. These tumors may arise wherever meninges exists, such as in the nasal cavity, paranasal sinuses, middle ear, and mediastinum. Optic nerve sheath meningiomas (ONSMs) are usually unilateral and occur predominantly in middle-aged females, although they may be present at any age. We present a case of a 55-year-old female with ONSM diagnosed when she was 40 years old. Diagnosis and follow-up was based on the clinical picture, CT orbit scan, and magnetic resonance imaging.

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery, 2020

Chordoid meningiomas (CMs) are a rare subgroup of tumors, accounting for ∼ 0.5% of all meningiomas. Chordoid meningioma tumors correspond to World Health Organization (WHO) Grade II lesions and behave aggressively, with an increased likelihood of recurrence. There are few genetic studies about CMs, but we understand that there is deletion at many chromosomal loci. Histologically, CMs are characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Morphologically, it can mimic other chondroid and myxoid tumors within the brain and its vicinity, thus posing a diagnostic challenge. Chordoid meningiomas have an aggressive clinical course and a propensity to recur compared with classical meningiomas. The goal of the treatment is surgery, with total resection of the tumor; however, due to its high degree of recurrence, radiotherapy is often necessary as an adjuvant treatment.

Indian Journal of Ophthalmology, 2014

A 60-year-old female presented with gradual, painless, progressive diminution of vision, and progressive proptosis of left eye since 7 years. Ophthalmological examination revealed mild proptosis and total optic atrophy in the left eye. Magnetic resonance imaging (MRI) and computed tomography (CT) brain with orbit showed bilateral optic nerve sheath meningioma (ONSM) involving the intracranial, intracanalicular, intraorbital part of the optic nerve extending up to optic chiasma and left cavernous sinus.

Neuropathology, 2004

Ş ş ş Chordoid meningioma is a rare variant of meningioma that bears a striking histological resemblance to chordoma and has greater likelihood of recurrence. Although most meningiomas occur in the intracranial, orbital and intravertebral cavities, rare meningiomas have been reported in extracranial organs; thus, it is important to be able to distinguish them from other neoplasms that have similar histology but different biological behavior and therapies. A case of chordoid meningioma in a 48-year-old woman who did not have Castleman's syndrome is described in the present report. The patient presented with a mass in her left frontoparietal region, and had been suffering from headaches for many years. Magnetic resonance imaging of the brain demonstrated an expansive lytic lesion in the squamous portion of the left temporal bone. The lesion extended in both directions. Histological examination of the surgical specimen revealed a tumor composed of cords and nests of eosinophilic vacuolated cells embedded in a myxoid matrix. A typical meningiomatous pattern was observed focally, and positive staining of the tumor cells for vimentin and epithelial membrane antigen confirmed the diagnosis of chordoid meningioma.

Romanian Journal of Neurology, 2019

Optic nerve sheath meningiomas (ONSM) are rare, frequently unilateral, benign, slowly growing tumors, arising from the meningo-epithelial cells surrounding the optic nerve intraorbital or intercanalicular. Their localization directly impair vision and indirectly impair aesthetics, making a safe classical surgical intervention very difficult and recommending stereotactic fractionated radiotherapy. Women are more affected, the survival rate is good and imaging can sustain differential diagnosis with optic neuroglioma or orbital schwannoma, lymphoma or pseudo tumor.

2014

SUMMARY Primary optic nerve sheath meningiomas (ONSM) are rare tumors of optic nerve which account for approximately 5-10% of orbital neoplasms. The classic triad of optic atrophy, progressive visual loss and presence of opticociliary shunt vessels is pathognomonic for the clinical presentation of ONSM however only a few percent of cases has this classic presentation. The most common presenting symptom in these patients is painless, progressive loss of visual acuity and dyschromatopsia. On clinical examination pathologic appearance of optic disc with varying degrees of optic atrophy is commonly observed. The goal of treatment for cases of ONSM is tumor control and improvement of vision. In cases with small and non-progressive tumor and high functional vision, conservative follow-up of the lesion is appropriate since surgical excision of ONSM is generally associated with high visual morbidities. Here we present a 67-year-old female patient with primary ONSM located on retrobulbar spa...

Chordoid meningioma (CM) is a rare subtype of meningi-oma, classified as grade II, which exhibits a high rate of recurrence following subtotal resection. We retrospectively examined nine cases of chordoid meningioma over a case series of 1743 meningiomas (0.52%) operated upon at our institution from 1995 to 2013. All the reported clinicopathological findings were analyzed. Two hundred and twenty-one CM cases have been published to date worldwide and few single-center large case series have been issued. Seventy-five percent of the cases that underwent subtotal resection at our institution had recurrence within 1 year. Total resection of the tumor should be the major objective of surgery to reduce the possibility of tumor recurrence. The percentage of chordoid features within the tumor specimen could assist in predicting the pathogenesis of the lesion. The correlation of the index of proliferation to recurrence rate is still controversial. Much debate exists with regard to the role of adjuvant radio-therapy in CM cases. Immunohistochemical, cytological and ultrastructural studies should be used in combination to assure a correct diagnosis of CM. Owing to the rare occurrence of this meningioma subtype, larger case series are required to assist in providing a reference for diagnosis and to improve the therapeutic management of CM.

Journal of neurological surgery reports, 2017

Optic nerve sheath meningiomas (ONSMs) account for less than 2% of meningiomas and 1.7% of orbital tumors. Although rare, the management of these tumors is important as unilateral blindness often results in untreated cases. Radiotherapy has emerged as the preferred treatment. However, therapies for ONSMs are controversial due to the variable natural history of the disease and limitations of surgical and radiotherapy options. A 60-year-old woman presented with monocular left diminished color perception and blurred vision. Magnetic resonance imaging demonstrated a homogenously enhancing 5-mm left optic nerve mass with evidence of nerve compression. Conservative management was advised. However, 1 month after diagnosis her visual acuity deteriorated further. Because of the small focal location of the tumor within the optic canal, surgery was considered. Given the tumor's location inferomedial to the optic nerve, an endoscopic endonasal approach to the optic canal was performed. This...

Journal of Neurosurgery, 2004

Object. The management of optic nerve sheath meningiomas (ONSMs) remains controversial but includes surgery, radiotherapy, and plain observation. Surgery is often thought to result in postoperative blindness. The authors report on a large series of patients surgically treated for ONSM, with an emphasis on the visual outcome. Methods. Seventy-three patients with ONSMs who had undergone surgery between 1991 and 2002 were retrospectively analyzed. The standard surgical approach consisted of pterional craniotomy, intradural (54 patients) or extradural (10 patients) unroofing of the optic canal, or a combined procedure (seven patients). Thirty-two tumors demonstrated extension through the optic canal. Twenty-nine tumors reached the chiasm or contralateral side. Patients with intraorbital flat tumors should undergo radiotherapy instead of surgery. Those with a large intraorbital mass and no useful vision should undergo surgery. Tumors extending intracranially through the optic canal are a...