Orbital malignant meningioma: a unique presentation of a rare entity

An elderly female with progressive proptosis was found to have an aggressive retrobulbar solid orbital mass. The mass was distinct from the optic nerve sheath and intracranial meninges, and produced concave erosion of the sphenoid wing. Operative findings demonstrated an orbital mass adherent to the dura of the superior orbital fissure. The mass did not demonstrate meningeal violation, infiltrate the superior orbital fissure, or display intracranial spread. The dura remained intact after gross total resection. Histopathology revealed a malignant meningioma with papillary and focal rhabdoid morphology and bony invasion (WHO grade III). The patient received 2500cGy of stereotactic radiotherapy in addition to gross total resection. Postoperatively, the signs and symptoms of orbital mass effect resolved (proptosis, relative afferent papillary defect, and periorbital edema) and the vision improved. There was no orbital recurrence or intracranial extension. The follow-up time was limited to eight months secondary to the patient succumbing to metastatic lung adenocarcinoma, which was demonstrated to be a separate process from the orbital meningioma. We propose the etiology of this tumor to be most consistent with an orbital malignant primary extradural meningioma – the first case reported in the literature.     

Orbital optic nerve gliomas in children with neurofibromatosis type 1

PURPOSE: To describe the clinical course and treatment of symptomatic orbital optic nerve gliomas in children with neurofibromatosis type-1 (NF-1). METHODS: A retrospective review of the records of patients with NF-1 and symptomatic orbital optic nerve gliomas seen in a large multidisciplinary NF-1 clinic of a tertiary care children's hospital. The main outcome measures included presenting symptoms and signs, ophthalmologic examination at diagnosis, the presence of progressive disease following diagnosis, type of therapy, and the reasons therapy was instituted. RESULTS: Twelve patients with symptomatic orbital optic nerve gliomas, all of which led to proptosis (eight girls, four boys), were identified. The mean age of diagnosis of NF-1 was 20 months; the mean age of diagnosis of the orbital optic nerve glioma was 26 months. At the time of diagnosis of the tumor, 10 of 12 patients (83%) had decreased visual acuity in the affected eye. Three patients underwent optic nerve resection; eight received chemotherapy, and one was observed without therapy. Of the eight children who received chemotherapy, progressive disease prior to treatment could be documented in only three; none of these eight children had a reproducible improvement in vision following chemotherapy. There was no demonstrable improvement in vision in any treated patient with NF-1-associated orbital optic nerve gliomas. CONCLUSIONS: Although not definitively proven, our data and previous studies suggest that NF-1-associated orbital optic nerve gliomas should not be treated unless there is clear evidence of either ophthalmologic or radiographic progression. Surgical excision of tumors which have led to proptotic eyes without functional vision should be reserved for cosmetic purposes or to treat complications of exposed globes.     

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.     

Orbital lymphoma mimicking optic nerve meningioma

purpose To describe two patients with orbital lymphoma mimicking optic nerve meningioma. methods Retrospective case review. results Both patients presented in their sixth decade with gradually increasing proptosis and decreasing vision. Computerized tomography revealed homogeneous masses encasing the optic nerves, which in conjunction with the clinical presentations were felt to represent optic nerve meningiomas. Biopsy, however, led to a diagnosis of lymphoma of mucosal-associated lymphoid tissue origin in one and a well-differentiated non-Hodgkin's lymphoma in the other. conclusion Orbital lymphoma surrounding the optic nerve may mimic the clinical and radiological appearance of an optic nerve meningioma.     

眶尖部及视神经肿瘤的早期诊断

目的探讨眶尖部及视神经肿瘤在眼球突出前的临床表现、影像学检查特征及临床诊断要点。方法回顾性分析22例无明显眼球突出单侧眼眶肿瘤患者的病历资料,包括主要症状、首发表现、临床诊治过程及影像学检查结果。结果22例肿瘤患者中,海绵状血管瘤6例,神经鞘瘤、视神经鞘脑膜瘤、鼻窦及鼻咽腔恶性肿瘤眶内蔓延各4例,后组筛窦黏液囊肿2例,神经纤维瘤和视神经胶质瘤各1例。以视力下降为首发症状者17例,视力下降合并复视者3例。全部患者均在CT或MRI检查后确诊。肿瘤位于眶尖部或起源于视神经。结论眶尖部及视神经肿瘤的早期症状是视功能障碍,无眼球突出表现。视力呈缓慢进行性下降,按视神经炎治疗效果差。CT或MRI检查对早期诊断起重要作用。     

Arachnoid cyst of the optic nerve: report of two cases and review of the literature

PURPOSE: To describe two patients with arachnoid cysts of the optic nerve mimicking optic nerve/orbital neoplasms. METHODS: The histories, ophthalmic examinations, and pathologic findings of two patients with arachnoid cysts of the optic nerves were reviewed and compared with previously reported cases. RESULTS: One patient had progressive proptosis and the other had decreased vision with visual field changes. Pathologic examination of optic nerve sheath biopsies showed redundant proliferations of meningothelial cells with associated thickened dura. CONCLUSIONS: Arachnoid cysts of the optic nerve are benign, slowly progressive conditions that may result in proptosis and visual field changes. The clinicopathologic features of arachnoid cyst should be differentiated from optic nerve sheath meningioma and other conditions.     

Unilateral optic neuritis,scleritis and exudative retinal detachment due to recurrent orbital pseudotumor

Idiopathic orbital inflammation, also known as orbital pseudotumor, describes a spectrum of idiopathic, non-neoplastic, non-infectious, space-occupying orbital lesions without identifiable local or systemic cause. The condition occurs mainly in young adults who may present with acute pain, proptosis, chemosis and limitations of extraocular movements. Decreased vision due to optic nerve infiltration and macular edema as a result of scleritis is less common sequel of orbital pseudotumor. Herein, we present a case of unilateral orbital pseudotumor in a young male who presented with chief complaints of painful decreased vision which was attributed to optic neuritis and macular edema as a result of scleritis. Imaging studies were helpful in the establishment of the correct diagnosis and patient’s complaints improved with administration of systemic corticosteroids.     

Prospective evaluation of fundus changes in patients with orbital mass lesions

PURPOSE: To study fundus changes associated with orbital mass lesions. METHODS: In a prospective, interventional case series, 28 patients undergoing surgery for discrete orbital masses had detailed fundus examination under dilation using direct and indirect ophthalmoscopy. Preoperative visual acuity and the amount and duration of proptosis were noted. Fundus photographs were taken, and postoperative changes were documented. The main outcome measures were the frequency of various fundus changes and their correlation with other clinical features. RESULTS: Nineteen (68%) of 28 patients had fundus changes; the commonest change was optic disk edema, seen in 14 patients (50%). The mean amount of proptosis +/- SD was 7.7 +/- 7.1 mm for patients with fundus changes and 4.22 +/- 2.1 mm for patients with normal fundus. Of 19 patients with fundus changes, 12 (63%) had an intraconal mass, and 11 (58%) had globe indentation. Twenty patients (71%) had diminished visual acuity preoperatively; of these patients, 8 (40%) had improvement in vision after surgery, 10 (50%) did not have any change in vision, and 2 (10%) had a postoperative decrease in visual acuity. Five (62%) of 8 patients with postoperative improvement in visual acuity had proptosis for < 2 years before surgery was undertaken, while only 1 (10%) of 10 patients retaining their preoperative vision had a duration of proptosis of < 2 years. CONCLUSION: Fundus changes are frequently seen with orbital masses. An increased amount of proptosis, intraconal location, and globe indentation are associated with fundus changes. Postoperative improvement in visual acuity is less likely in patients with a longer duration of symptoms.     

60例眶颅沟通肿瘤的诊断分析

目的探讨眶颅沟通肿瘤的临床特征、种类及诊断方法。方法选择60例手术后经病理或随访结果证实的眶颅沟通肿瘤患者,所有患者均行CT扫描,54例行MR I扫描,其中增强扫描42例。20例摄视神经管X光像。结果最常见的阳性体征是眼球突出(58/60)、眶压升高(39/60)和视力下降(36/60),最常见的症状是头痛(22/60)。较多见的眶颅沟通肿瘤是脑膜瘤(20/60)、骨纤维异常增殖症(5/60)和转移癌(5/60)。眶颅沟通肿瘤的CT、MR I扫描及视神经孔X光像有一定影像学特征。眶颅沟通途径包括:视神经管、眶上裂和眶颅交界骨壁。结论掌握眶颅沟通肿瘤的临床特征、病种分类,综合运用CT、MR I和X光等检查方法可提高诊断和鉴别诊断水平。     

Orbital decompression for thyroid orbitopathy

Background: Severe thyroid orbitopathy may result in optic neuropathy, corneal exposure and disfiguring proptosis, Orbital decompression has most commonly been performed for optic neuropathy, but with improved techniques, more patients are undergoing decompression for other indications. Purpose: This report evaluates the results and morbidity of orbital decompression for thyroid orbitopathy performed by one surgeon. Methods: The records of 33 patients (53 orbits) undergoing orbital decompression for thyroid orbitopathy were analysed for changes in visual acuity and colour vision (where the indication was optic neuropathy) and reduction in proptosis. Complications were also analysed. Results: Visual acuity and colour vision improved in all 33 eyes with optic neuropathy in the short term postoperative period (4 weeks), but later deteriorated in five eyes (6.6%) of 4 patients (19%). Proptosis decreased by a mean 5.3 mm (range, 1–10). Diplopia developed or worsened overall in 10 of 33 patients (30%), but only in one of 12 (8%) where the indication was cosmesis or corneal exposure. Diplopia improved in 2 of 33 (6%). All patients with symptomatic diplopia achieved binocular single vision in a useful range after one and sometimes two squint procedures. No patient lost vision as a result of surgery. Conclusions: Orbital decompression is effective in improving vision in most patients with thyroid optic neuropathy, but induces or worsens diplopia in a high proportion of these patients. Proptosis can be effectively and dramatically improved.     

Intraosseous meningioma: a rare cause of chronic optic neuropathy and exophthalmos

BACKGROUND: The association of optic neuropathy, proptosis and chorioretinal folds is highly suggestive of an orbitopathy. Usual etiologies include an inflammatory orbitopathy, optic nerve sheath meningioma, sphenoid wing meningioma, exterioration of a sinus disorder, or an orbital tumor be it primary or secondary. HISTORY AND SIGNS: A 40-year-old man treated for systemic hypertension complained of decreased vision and floaters in his right eye. Initial examination revealed decreased visual acuity to 20/50 of the right eye with a slight dyschromatopsia, but a lack of afferent pupillary defect and normal visual fields. Fundus examination showed the presence of a slightly swollen right optic disc and chorioretinal folds. A diagnosis of presumed anterior ischemic optic neuropathy was made. Symptoms persisted and, five months later, right proptosis was noted. Magnetic resonance imaging revealed a diffuse thickening of the parieto-temporal bone and the greater wing of the sphenoid bone on the right side. Radiological differential diagnosis included fibrous dysplasia and metastasis. THERAPY AND OUTCOME: Bone biopsy revealed a grade I intraosseous meningioma. Conservative management was chosen because the lesion was too extensive to be resected and radiotherapy is usually not efficient on grade I meningiomas. CONCLUSIONS: Intraosseous meningiomas are benign tumors which are due to meningeal cells entrapment during vaginal delivery. It is a rare tumor of slow progression. Therapy usually consists of resection and cranioplasty and/or radiotherapy. In the present case, decompression of the optic canal remains feasible in case of further visual loss.     

Endoscopic transnasal orbital decompression for visual failure due to sphenoid wing meningioma

AIM: To review a group of patients with sphenoid wing meningioma and visual impairment. The technique and results for endoscopic transnasal decompression of the orbital apex is presented. PATIENTS AND METHODS: Patients presenting between 1993 and 2004 with visual impairment due to sphenoid wing meningioma were identified. All patients had full ophthalmic assessment, automated visual field testing, and appropriate orbital imaging. Patients with clinical evidence of significant progression in visual impairment (loss of acuity, reduced colour discrimination, or field deterioration) underwent endoscopic transnasal orbital decompression. OUTCOME MEASURES: Assessment of visual function (Snellen acuity, Ishihara colour perception, and visual field testing), together with other measures of orbital structure and function (ocular balance, ductions, and exophthalmometry). RESULTS: Twelve patients (all women) presented to the Orbital Clinic between the ages of 38 and 71 years (mean 42.6; median 48.5 years) and with symptoms for an average of 32 months (3-102 months; median 22). The most common symptom was swelling and proptosis, in 11 (91%) patients, and subjective deterioration of vision had been noted in six cases (50%). Eight right orbits (67%) were affected and the preoperative acuity varied between Snellen 6/5 and counting fingers, with visual field impairment in all cases. There was an average of 5.0 mm of axial proptosis (range 5-9 mm; median 5).Endoscopic decompression was without complication in all cases. With a follow-up interval of 33.9 months (range 5-80; median 26 months), there was a subjective and objective improvement in visual functions-with Snellen acuity improving between 1 and 4 lines in seven patients, reduction in relative afferent pupillary defect in 10/12 patients, and improved visual field testing. There was a 2.3 mm reduction in proptosis (1-4 mm; median 3 mm) with less lid swelling, improved ocular balance and motility in four patients, and slightly worse diplopia in one patient.Three patients required further procedures: one had ipsilateral middle meatal antrostomy for retained secretions at 18 months and two had strabismus surgery. Three patients underwent fractionated radiotherapy for large tumours, or for late tumour growth and recurrent visual impairment. CONCLUSION: Orbital decompression by transnasal endoscopic ethmoidectomy appears to alleviate optic nerve compression due to sphenoid wing meningioma, with a reasonable relief of the condition for some years. Some patients will require later radiotherapy for progressive tumour growth or visual failure.     

Gasserian ganglion schwannoma with orbital extension

A patient with painless proptosis and no associated neurologic symptoms was found to have a cystic retrobulbar orbital mass. The initial computed tomography (CT) scan did not demonstrate any intracranial involvement. On orbital biopsy a schwannoma was encountered and repeat CT scan with contrast revealed a large middle cranial fossa mass with extension into the orbit through the optic canal and superior orbital fissure. On lateral transfrontal craniotomy the lesion was noted to arise from the gasserian ganglion and to involve the right cavernous sinus. Subtotal resection was performed, and the patient has remained asymptomatic and free of recurrence for 18 months.     

Progressive visual loss. What type of mass lesion?

A 43-year-old woman presents with painless loss of vision in her left eye. She has decreased visual acuity, an afferent pupillary defect, slight proptosis and disc swelling, implying an optic nerve lesion. Computerized tomographic scanning shows generalized enlargement of the posterior orbital optic nerve. A presumed diagnosis of an optic nerve sheath meningioma is made. The discussion deals with the problems of rational treatment.     

Orbital liposarcoma: a retrospective, single-center study of thirteen patients

AIM: To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma. METHODS: The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital, from 2006 to 2021 were collected and analyzed. The data includes age, gender, affected orbital side, course of disease, status of disease (primary or recurrent), clinical manifestations, preoperative visual acuity, operative treatment, the relations between liposarcoma and surrounding tissue, longest diameter of liposarcoma, histological subtype, immunohistochemical indicators, follow-up treatment and prognosis. RESULTS: The initial symptoms are diverse. Proptosis is the most frequent chief complaint and the others included vision loss, epiphora, diplopia, and eyelid palpable mass. Results of imaging examination [computed tomography (CT) or magnetic resonance imaging (MRI)] showed orbital mass. In terms of treatment, 10 patients received tumor resection, and the mean longest diameter of the tumor was 3.39±1.36 cm. The other 3 patients had optic nerve invaded, so they received orbital exenteration. Pathological examination results confirmed the diagnose of liposarcoma for 13 patients. Six patients displayed as myxoid type, and three patients in each type of dedifferentiated and well-differentiated type. One patient was verified as pleomorphic, which was a rare type of liposarcoma. All of the patients showed Vimentin positive, and most showed CD34 and S-100 positive. Besides, four patients showed smooth muscle actin positive. All thirteen patients were alive. CONCLUSION: Orbital liposarcoma is a rare disease and it has no specific clinical manifestation. The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit. It is recommended to perform pathological examination to achieve early detection and early treatment.     

Apical involvement with fibrous dysplasia: implications for vision

PURPOSE: To describe the ophthalmic findings of fibrous dysplasia of the orbit. METHODS: Prospective case series. Twenty-one patients with fibrous dysplasia with orbital involvement underwent a complete ophthalmic examination and orbital imaging by CT. RESULTS: Four of the 21 patients had McCune-Albright syndrome and 1 had tuberous sclerosis. In 17 patients (81%), the disease was restricted to the craniofacial region. Facial distortion, proptosis, and eye dystopia were detected in 62% of the patients. Nasolacrimal duct obstruction and strabismus were detected in only 1 and 2 patients, respectively. Of the 34 orbits affected, the roof was affected in 67.6%. The floor was the least affected wall. For most orbits, the disease was seen in more than 1 wall, and in 9 orbits (26.5%) all 4 walls were involved. Twelve orbits (35.2%) showed cysts on CT within the affected bones. In 19 orbits (55.9%), the optic canal was circumferentially narrowed. Blindness was detected in only 1 patient who had undergone optic canal decompression. CONCLUSIONS: The main consequences of orbital involvement in fibrous dysplasia are eye dystopia and proptosis. Apical involvement is a common feature. Optic canal narrowing does not necessarily induce vision loss. We believe that patients with apical involvement should be carefully followed and have their apices decompressed only when their vision is clearly deteriorating. Our data do not support prophylactic optic canal decompression as a therapeutic measure.     

Hemangioblastoma of the optic nerve

Optic nerve hemangioblastoma is a rare tumor that is usually unilateral and most commonly occurs in the context of von Hippel-Lindau disease. Differential diagnosis is based on clinical history and imaging. Magnetic resonance imaging with gadolinium enhancement is the most useful imaging modality as it can reveal flow voids and an absence of dural attachment, differentiating optic nerve hemangioblastoma from other more commonly encountered optic nerve tumors. Optic nerve hemangioblastoma are usually well-circumscribed vascular lesions composed of stromal cells and vascular endothelial cells. These lesions are diagnosed at a mean age of 37 years and can be asymptomatic, but over time, patients may develop reduction in vision, proptosis, and pain. Surgical excision is well described via orbital, transsphenoidal, or transcranial approaches. Given the risks associated with surgery, a stepwise conservative approach is advocated by most clinicians in the absence of severe symptoms. Although uncommon, this optic nerve tumor should be considered in young patients presenting with pain, proptosis, and optic nerve pallor, with or without a history of von Hippel-Lindau disease.     

甲状腺相关眼病眼眶减压术的疗效分析

目的：探讨眼眶减压术在甲状腺相关眼病中治疗的价值。方法：回顾性分析中山眼科中心1993－2000年27例（30只眼）经全身和眼部临床检查（视力、视野或视觉诱发电位等）确诊为甲状腺相关眼病患者采用眼眶减压术（一壁、二壁及三壁减压）治疗的临床资料,观察其手术前和手术后患者视力、眼球突出度及眼球运动的变化。术后随访2个月至7年,平均13．7个月。结果：视力：19只眼（63．3％）明显提高;4只眼（13．3％）轻度提高,视力均保持在0．2－0．8;4只眼（13．3％）视力无变化,其中3只眼（10．0％）视力下降。24只眼（80．0％）眼球突出后退≥3．0mm,28只眼（93．3％）眼球突出后退≥2．0mm,平均眼球突出后退3．6mm。结论：眼眶减压术可提高甲状腺相关眼病患者的视力,减轻其眼球突出度。     

Evolution of an orbital apex tumor over a decade

We report the evolution of an orbital apex tumor that underwent spontaneous regression, followed several years later by rapid growth and loss of vision. A turkey farmer had a right apical lesion, initially thought to be an optic nerve meningioma. The mass spontaneously regressed over 2 years, with marked improvement of vision. Eight years later, the patient presented with loss of light perception and severe proptosis of the right eye from a massive orbital tumor that extended in the cavernous sinus. An eyelid-sparing orbital exenteration was performed. The pathologic diagnosis was diffuse large cell lymphoma. This case demonstrates that non-Hodgkin lymphoma of the orbital apex may resolve spontaneously or it may enlarge rapidly, degenerating into more aggressive forms.     

Is primary optic nerve sheath schwannoma a misnomer? Report of two cases and literature review

Aims: To report clinicopathological characteristics of two patients with optic nerve sheath schwannoma (ONSS) and review the literature.

Method: The first patient (22-year-old man) presented with left eye proptosis and decreased vision in 2012 whose orbital imaging showed a large cystic lesion around the optic nerve. The second patient (52-year-old man) presented with decreased vision in the left eye (without proptosis) in the 2006. His imaging showed a small orbital apex lesion between the medial rectus and optic nerve. Both lesions were histopathologically consistent with ONSS. ONSS has previously been reported in 12 patients.

Results: Orbital biopsy and subsequently external beam radiotherapy were performed for the first patient who showed a temporary improvement of vision. However, his proptosis progressed and vision decreased to light perception (LP) a few months after radiotherapy when the lesion was resected in April 2013. Second patient declined any procedure until his vision gradually decreased to LP in October 2007. It was then removed through a combined medial and lateral orbitotomy procedure. Both patients ended up with visual acuity of no LP and no recurrence in their last follow up visits in April 2018. No histopathological evidence was found to show that optic nerve sheath could be the origin for presenting and previously reported cases in the literature.

Conclusion: Presenting cases and literature review imply that ONSS is a misnomer and all the lesions are different types of orbital schwannomas with optic nerve sheath attachment.