Ectopic orbital meningioma: a case report and review

PURPOSE: To describe a well-documented case of ectopic orbital meningioma, to review the literature on the subject, and to recommend treatment. METHODS: Neuroradiologic investigations were suggestive of orbital meningioma. The patient underwent total excision of the mass, which was subsequently examined by light microscopy and immunohistochemistry. RESULTS: The patient was diagnosed with ectopic orbital meningioma which was locally invasive. The tumor was surgically excised, and did not recur during a 42-month follow-up interval. CONCLUSIONS: Ectopic orbital meningiomas are rare tumors that develop from ectopic arachnoid tissue. Although locally invasive, the prognosis is favorable if the tumor is completely removed.     

Suprasellar meningioma 47 years after bilateral retinoblastoma

Development of an additional primary tumour, a suprasellar meningioma, is reported 47 years after successful treatment of bilateral retinoblastoma. The left eye of the patient was enucleated at the age of 1.5 years and the left orbit radiated with a total dose of 1200 rads. The right eye was treated by orthovoltage radiation only at the age of 4 years, the total dose being 5100 rads. The right eye became blind and phthisical, and it was enucleated at the age of 42 years. No active retinoblastoma was found. At the age of 48 years a large suprasellar meningioma was diagnosed and partially removed.     

Optic Nerve Meningioma

An obese white woman presented with a 10-month history of progressive loss of vision in the right eye associated with a moderate proptosis. Examination of the fundus revealed marked congestion of the retinal vasculature with dilation of both the arteries and the veins. Numerous blot hemorrhages were scattered throughout the retina. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography demonstrated only a serous detachment of a thickened, markedly convoluted retina. A diagnosis of optic nerve meningioma was considered, but definitive treatment was deferred because of medical problems. One month later the patient returned with a blind, painful right eye associated with neovascular glaucoma. High resolution CT coronal sections of the orbit showed generalized thickening of the optic nerve. The blind eye was enucleated facilitating exposure of the optic nerve which was found to be diffusely thickened by meningioma. The orbital portion of the optic nerve was subsequently removed and meningioma was demonstrated along its entire length to the bony optic canal. A transfrontal craniotomy was later performed to rid the patient of residual tumor.     

Sugarplum fairies. Visual hallucinations

A 35-year-old woman with a history of early childhood encephalitis and a cerebrovascular accident during childbirth at 29 presented with visual hallucinations of two years' duration. A meningioma was removed from the right middle cranial fossa, and the visual hallucinations ceased. Visual hallucinations are complex phenomena which may arise through stimulation of the visual pathways at many levels. The case report is followed by discussion of various types of visual hallucinations and the theories and evidence regarding their pathophysiology.     

Intraocular meningothelial meningiomas

A 24-year-old male was referred for evaluation and treatment of a choroidal tumor located 1 1/2 disc diameters from the optic disc of the right eye. The tumor had doubled in size over a 29-month period of observation, and measured 8 × 8 × 5 mm. The intraocular tumor was removed by internal eye wall resection, and the pathology specimen revealed the tumor to be a meningothelial meningioma.     

应用γ刀治疗眼眶脑膜瘤临床观察

目的 观察γ刀在眼眶脑膜瘤治疗中的作用和适应证.方法 对43例经γ刀治疗的眼眶脑膜瘤患者进行定期随访,其中34例得到临床和影像学随访结果;γ刀放射治疗平均中心剂量32.22 Gy,等剂量曲线范围为79%～95%,平均随访时间30个月.结果 治疗后肿瘤缩小19例,消失2例,无变化6例,增大3例,4例未做影像学检查;肿瘤控制率为90.0%（27/30）.治疗后视力提高12例,减退4例,丧失3例,不变15例.除1例因脑血管疾病死亡外,无死亡病例.结论 γ刀治疗对眼眶脑膜瘤是一种有效的微创治疗方法,并发症少,可保留一部分视力,适用于视神经鞘脑膜瘤向视神经管内蔓延、眶内异位脑膜瘤及蝶骨嵴脑膜瘤.     

Malignant (anaplastic) transformation of orbital clear cell meningioma

A 69-year-old woman with a history of a previously resected right sphenoid wing meningioma and radiation presented with a 4-month history of a rapidly progressive right orbital swelling. MRI revealed a sphenoid wing mass extending in the right orbit and depressing the right globe. An incisional biopsy revealed a clear cell meningioma with anaplastic features. Histopathologic examination showed that the tumor was composed of sheets of polygonal cells with clear cytoplasm consistent with clear cell meningioma. Nuclear atypia with a high mitotic activity was also evident; thus, the tumor was classified as anaplastic. Clear cell meningioma is a rare form of meningioma usually located in the cerebellopontine or spinal cord areas.     

Unilateral acquired upper eyelid ptosis and decreasing nystagmus

PURPOSE: To describe a patient with congenital nystagmus that decreased in one eye after the development of a cavernous sinus meningioma. DESIGN: Interventional case report. METHODS: A 45-year-old man with congenital nystagmus, albinism, and high myopia presented with right upper eyelid ptosis that was corrected surgically on three occasions during a 3-year period. An episode of syncope was followed by diagnostic evaluation that disclosed a cavernous sinus meningioma. He commented that his right eye nystagmus progressively decreased. He was found to have ophthalmoplegia on the right eye due to nerve compression by the meningioma. RESULTS: A magnetic resonance imaging scan disclosed a cavernous sinus meningioma extending into the parachiasmal and clival areas. A biopsy specimen of the lesion demonstrated a meningioma. CONCLUSIONS: Any patient with acquired ptosis should have a complete ophthalmic examination, including evaluation of extraocular motility.     

原发于眼眶脑膜瘤的CT图像分析

目的分析原发于眼眶脑膜瘤的常见CT图像特征,探讨CT影像对原发于眼眶脑膜瘤的诊断价值。方法回顾性分析51例经病理确诊的原发于眼眶脑膜瘤患者的临床资料和CT图像表现。探讨CT对脑膜瘤的诊断及鉴别诊断价值。结果 51例原发于眼眶脑膜瘤患者中视神经鞘脑膜瘤43例,眶骨膜脑膜瘤8例。视神经鞘脑膜瘤43例中肿瘤形状呈管状20例,块状12例,其他形状11例;呈现车轨征16例,钙化斑6例。8例眶骨膜脑膜瘤均有蝶骨大翼和(或)小翼骨膜增厚及眶外壁骨质增生,其中视神经向内移位6例。所有病例中肿瘤蔓延者8例,双眼眶发病2例。结论 CT图像可清晰显示肿瘤的位置、形状、范围,尤其是眶骨壁改变,对于原发于眼眶脑膜瘤的诊断及治疗具有重要的临床指导意义。     

Ectopic orbital meningioma: report of two cases and literature review

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.     

A sphenoid en plaque meningioma aggravates exophthalmos in a patient with thyroid ophthalmopathy

PURPOSE: To describe the rare case of a patient with thyroid ophthalmopathy whose unilateral aggravated exophthalmos was caused by the development of an ipsilateral sphenoid wing en plaque meningioma. METHODS: Case report. RESULTS: The ophthalmologic examination included visual acuity assessment, anterior segment examination, funduscopy, ocular motility examination, and exophthalmometry. Magnetic resonance imaging (MRI) of the brain and orbit revealed the existence of a sphenoid meningioma. Removal of the tumor through a pterional craniotomy was performed. The histologic examination showed that it was an en plaque meningioma. There were no postoperative complications and no recurrence of the tumor was revealed 24 months after the operation. CONCLUSIONS: Detailed ophthalmologic examination and MRI of the brain and orbit are necessary in every patient with deterioration of the exophthalmos even if the suggestive cause seems obvious.     

原发性眼眶内脑膜瘤的临床及病理,免疫组化观察

目的 探讨眼眶内原发性脑膜瘤的临床、病理及免疫组化特点。方法 回顾性分析１５例眶内脑膜瘤的临床资料，复习症理切片及免疫组化结果。结果 患者男６例、女９例，４￣５２岁（平均３３．３岁）。主要临床表现为眼球突出、视力严重受损及视神经萎缩。大体病理形态与颅内脑膜瘤相同。２例侵犯眼眶骨质，但核分裂相少。免疫组化阳性率为：ＶＩＭ１００％，ＥＭＡ６０％，ＣＫ４６．６７％，Ｓ１００４０％，ＮＳＥ６０％，ＧＦＡＰ     

Ectopic orbital meningioma: Fact or fiction?

Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.     

Optic nerve sheath meningioma: visual improvement during radiation treatment

PURPOSE: To describe four patients with optic nerve sheath meningioma in whom visual improvement began to occur even before completion of a standard course of radiation therapy. DESIGN: Retrospective case review. METHODS: A review was conducted of 35 patients who received stereotactic three-dimensional conformal radiotherapy for optic nerve sheath meningioma from 1990 to 2005. RESULTS: Four patients with radiographic confirmation of optic nerve sheath meningioma were identified who had neuro-ophthalmologic assessment, which included perimetry, either while radiation treatment was underway or immediately afterwards. All of the patients showed improvement in visual acuity and visual fields. CONCLUSION: A rapid response to radiation therapy may occur in some patients with optic nerve sheath meningioma. In such patients, it may be possible to customize the radiation dose by assessing of visual function during the course of therapy.     

Orbital meningioma,the Utrecht experience

AIMS. 1) To evaluate epidemiological data (age, gender, initial complaints, and ophthalmic findings) of a patient cohort with a primary or secondary orbital meningioma. 2) To evaluate the clinical course of these patients. 3) To evaluate the outcome of treatment. PATIENTS AND METHODS. All consecutive patients with a presumed meningioma with orbital involvement seen at the Academic Medical Center, Utrecht, in the period 1/1/1992-31/12/1999 were evaluated retrospectively. RESULTS. Sixty-three patients with either an optic nerve sheath (n = 16) or a sphenoid ridge or tuberculum sellae meningioma (n = 47) were seen (mean age: 41.9 and 47.6 years, respectively); 20 of these had been treated neurosurgically previously. Fifty-three were females. The most frequent initial symptoms in both groups were proptosis and visual complaints. Thirty-three patients were followed without treatment, eight of them showing a lingering worsening of vision and a slow increase of tumor mass. Thirty patients were operated for different reasons (to confirm the diagnosis, or because of decreasing vision, disfiguring proptosis, threatening of the optic chiasm, or severe retrobulbar pain). Life-threatening problems did not occur, cranial nerve damage being the most frequent complication. Macroscopic radical tumor resection was only obtained in about 50% of the operated patients, but immediate (partial) relief of subjective complaints was obtained in up to 90%. A recurrence with clinical symptoms was seen in two patients within the relatively short follow-up period. CONCLUSIONS. Proptosis and, secondly, vision complaints are the most frequent symptoms in patients with either a primary or a secondary orbital meningioma. Their clinical course is extremely variable. Loss of vision is frequently seen in both groups. Orbitoneurosurgical meningioma resection has a high immediate success rate. Damage to cranial nerves is the most frequent complication of meningioma resection.     

Metastatic malignant meningioma

The onset of a rapidly progressive abducens and trigeminal neuropathy, third-order neuron Horner's syndrome, and decreased lacrimation clinically suggest a malignant lesion at the base of the middle cranial fossa, commonly a metastatic process. A case is reported in which computed tomography and magnetic resonance imaging failed to image the lesion but a bone scan clearly depicted the abnormal area. A malignant meningioma (en plaque) was evident on biopsy, and pulmonary metastases later ensued. Common histological patterns of meningioma (often thought of as a benign tumor) include meningothelial, fibrous, and transitional types. The association of cellular atypia, nuclear pleomorphism, marked mitoses, and brain invasion warrants the designation of malignant meningioma. The incidence of malignancy in meningioma ranges from 2 to 10% with reported metastases occurring in 0.1%.     

Successful use of mitomycin C to prevent recurrence of the cystic component of an optic nerve sheath meningioma

A 28-year-old woman presented with 4 months of episodic right-eye vision loss and proptosis. Imaging demonstrated a cystic retrobulbar lesion. Lateral orbitotomy with drainage and biopsy revealed a cystic optic nerve sheath meningioma. The cyst recurred despite radiation therapy followed by craniotomy with partial resection of the meningioma. In an effort to preserve vision, an anterior orbitotomy for cyst drainage with topical mitomycin C was performed. Since this procedure, the cyst and symptoms have remained stable for 19 months. We report a case of cystic optic nerve sheath meningioma and the successful treatment of cyst recurrence with mitomycin C.     

Oculomotor Nerve Palsy Associated with Duplication of Middle Cerebral Artery,Anterior Communicating Artery Aneurysm,and Parietal Meningioma

A 61-year-old male presents with diplopia of acute onset and progressive course. He has a history of previous intracranial haemorrhage that was surgically evacuated 7 years ago and was also associated with diplopia. Examination revealed left complete oculomotor nerve paralysis with a fixed and dilated left pupil. Computed tomography (CT) revealed encephalomalacia, evidence of previous craniotomy, and an incidental left parietal convexity meningioma. CT angiography of the brain revealed a left tortuous duplicate middle cerebral artery with fenestration of its proximal part, an anterior communicating artery aneurysm, and a characteristic capillary blush of the meningioma. Possible mechanisms of oculomotor nerve involvement are discussed.     

Vogt-Koyanagi-Harada syndrome with intracranial meningioma: an as yet unreported association

CASE REPORT: We document a previously unreported association of Vogt-Koyanagi-Harada (VKH) syndrome with intracranial meningioma. A female patient with diminished vision, exudative retinal detachment, and headache was diagnosed with VKH syndrome, more precisely a Harada form of disease with intracranial meningioma, on the basis of exudative retinal detachment, typical fundus fluorescein findings, and magnetic resonance imaging. With intravenous steroid therapy, visual acuity improved and the detachment settled within a week. At 3 months, the detachment recurred but improved after retreatment. At 1 year, the tumour was unchanged in size. COMMENTS: VKH syndrome may be associated with intracranial meningioma that may affect the patient's overall morbidity or mortality.     

Arachnoid hyperplasia in optic nerve glioma: confusion with orbital meningioma.

A case is reported of an optic nerve glioma with a marked degree of arachnoid hyperplasia which was initially diagnosed as an optic nerve meningioma. Hyperplasia of the arachnoid was also the underlying cause for expansion of the optic canal. The relationship between arachnoid hyperplasia in optic nerve glioma and meningioma of the optic nerve sheath in childhood is discussed.