Optic glioma of childhood: clinical, histopathological, and histochemical observations.

This report documents the long-term clinical and histopathological behaviour of eight intraorbital and 16 intracranial optic nerve gliomas and relates the therapeutic data to the prognosis for both visual acuity and survival. The mean age at onset of symptoms was 8.6 years and at the diagnosis 10.9 years. It is generally held that proptosis is mild in intraorbital glioma, but we encountered marked proptosis ranging from 7 to 12 mm in six of the eight intraorbital gliomas, which contained abundant Alcian-blue-positive mucoid material. Of the patients with intracranial optic nerve gliomas 37.5% survived for a mean of eight years after treatment with radiotherapy or surgery combined with radiotherapy. At follow-up ranging from five months to 11 years only one of the six patients with intracranial gliomas had full visual acuity. Our observations emphasise that, although optic nerve gliomas are benign hamartomas, the prognosis for visual acuity and survival is unfavourable in cases which are diagnosed and treated late. Histopathological and histochemical observations suggest that increase in the amount of mucoid material may contribute to rapid enlargement of intraorbital and intracranial optic gliomas.     

Acute visual loss due to a calcified optic nerve glioma

Optic nerve gliomas are slow-growing tumours most commonly seen in children under 10 years of age. Rapidly progressive proptosis and rapid visual deterioration are uncommon but may occur owing to accumulation of mucoid material, necrosis or hemorrhage. We describe a patient with an optic nerve glioma who manifested sudden proptosis and blindness caused by hemorrhage within the optic nerve sheath. The visual acuity returned to 20/25 after surgical decompression of the nerve and high-dose steroid therapy. Histopathological examination was required to establish the diagnosis of optic nerve glioma with extensive calcification. Optic nerve decompression or short-term high-dose steroid therapy, or both, may be helpful in recovering visual function in selected patients with optic nerve gliomas who have acute visual loss.     

Aggressive Low-Grade Optic Nerve Glioma in Adults

Abstract

Primary optic nerve gliomas are most commonly benign pilocytic astrocytomas (World Health Organization [WHO] Grade I) occurring in childhood and following an indolent course. Malignant optic gliomas occur in adulthood and follow an extremely aggressive course, with rapid infiltration of the chiasm, blindness, and death typically within months. A third category of optic glioma, occurring in adulthood, histopathologically benign (WHO Grade I–II) but following an aggressive course, has been rarely reported. The authors describe clinical and histopathologic features of clinically aggressive but histopathologically benign optic nerve gliomas of adulthood. Retrospective review of cases of biopsy-proven optic nerve glioma in the neuro-ophthalmology division of the Jules Stein Eye Institute from 1990 to 2011 was carried out. Cases following an aggressive course were selected for review of clinical, neuroradiologic, and histopathologic features. Three cases were selected for detailed study. Ages ranged from 31 to 45 years. All were initially diagnosed with optic nerve inflammation or benign neoplasm based on clinical and neuroradiologic features, but all suffered neuroradiologic extension and rapid deterioration of vision in the affected eye to no light perception over 3–8 weeks. Optic nerve biopsies were undertaken for the suspicion of malignancy. Features ranged from WHO Grade I (pilocytic astrocytoma, ganglioglioma) in two cases, to WHO Grade II in one case (diffuse astrocytoma, histopathologically benign, but associated with aggressive features such as high p53 [13–21%] and Ki-67 [40%]). The diffuse astrocytoma case subsequently developed extensive intracranial extension suspicious for malignant transformation. These findings indicate that benign optic nerve glioma in adults may be initially misdiagnosed as inflammation, be clinically aggressive, and require excision to prevent further intracranial involvement.     

Aggressive Low-Grade Optic Nerve Glioma in Adults

Primary optic nerve gliomas are most commonly benign pilocytic astrocytomas (World Health Organization [WHO] Grade I) occurring in childhood and following an indolent course. Malignant optic gliomas occur in adulthood and follow an extremely aggressive course, with rapid infiltration of the chiasm, blindness, and death typically within months. A third category of optic glioma, occurring in adulthood, histopathologically benign (WHO Grade I–II) but following an aggressive course, has been rarely reported. The authors describe clinical and histopathologic features of clinically aggressive but histopathologically benign optic nerve gliomas of adulthood. Retrospective review of cases of biopsy-proven optic nerve glioma in the neuro-ophthalmology division of the Jules Stein Eye Institute from 1990 to 2011 was carried out. Cases following an aggressive course were selected for review of clinical, neuroradiologic, and histopathologic features. Three cases were selected for detailed study. Ages ranged from 31 to 45 years. All were initially diagnosed with optic nerve inflammation or benign neoplasm based on clinical and neuroradiologic features, but all suffered neuroradiologic extension and rapid deterioration of vision in the affected eye to no light perception over 3–8 weeks. Optic nerve biopsies were undertaken for the suspicion of malignancy. Features ranged from WHO Grade I (pilocytic astrocytoma, ganglioglioma) in two cases, to WHO Grade II in one case (diffuse astrocytoma, histopathologically benign, but associated with aggressive features such as high p53 [13–21%] and Ki-67 [40%]). The diffuse astrocytoma case subsequently developed extensive intracranial extension suspicious for malignant transformation. These findings indicate that benign optic nerve glioma in adults may be initially misdiagnosed as inflammation, be clinically aggressive, and require excision to prevent further intracranial involvement.     

Optic glioma with intraocular tumor and seeding in a child with neurofibromatosis

We treated a 3-year-old boy with neurofibromatosis who had an optic glioma, intraocular extension with seeding, and iris tumors. On the basis of results of ultrasonography, computed tomography, magnetic resonance imaging, and fine needle aspiration, other intraocular and orbital tumors were excluded. Because of the malignant intraocular aspect, the optic nerve glioma was extirpated. Histologic examination confirmed the diagnosis of an optic nerve glioma with intraocular extension, seeding, and iris nodules. In this juvenile pilocytic astrocytoma with secondary perineural fibrous hyperplasia, several mitoses were found in the orbital and intraocular parts. In the optic canal, three small islands were found that were compatible with the diagnosis of malignant astrocytoma, grade 3. The iris nevi appeared as iris pits and not like the Lisch nodules typical of neurofibromatosis. Café au lait spots were present on the skin. The family history was positive for neurofibromatosis. The results of this study demonstrate that optic gliomas are true astrocytomas and not hamartomas, and have a continuous scale from benign to malignant differentiation.     

Malignant gliomas of the optic nerve pathways

We compared the clinical course and histopathologic findings in five patients with malignant gliomas originating from the optic nerve pathways with those in previously reported cases to elucidate further the clinical features of this tumor and facilitate future diagnoses. Patients with malignant optic nerve pathway gliomas had unilateral or bilateral visual dysfunction, which was often accompanied by periorbital discomfort in an otherwise asymptomatic adult. Regardless of origin, the clinical course invariably was bilateral blindness rapidly followed by death. Occasionally, the diagnosis was made preoperatively and was confirmed by surgical exploration and biopsy.     

视神经胶质瘤的诊治研究现状及进展

视神经胶质瘤（ONG）是一种好发于儿童和青少年且相对罕见的中枢神经系统肿瘤,主要病理类型为低级别的毛细胞型星形细胞瘤。其分为散发和1型神经纤维瘤病（NF-1）相关的ONG。由于ONG与视神经的紧密关系,在诊疗上存在其特殊性,其诊断主要依靠病史、症状和体征,以及磁共振成像和CT等影像检查。ONG应与神经鞘脑膜瘤、视神经炎...     

Acute visual loss: just the beginning?

A 47-year-old man presented with sudden visual loss, optic disk edema, retinal ischemia, and limited upgaze in the left eye. Initial MRI revealed thickened, enhancing left optic nerve. Extensive work-up for an inflammatory and infiltrative etiology was positive only for Borrelia burgdorferi IgM by Western blot. Six weeks later the patient had numbness and weakness on his left side. MRI showed enhancing lesions extending from the left optic nerve to the optic chiasm, along the visual pathways bilaterally, mainly on the right side from optic tract to lateral geniculate body and pulvinar. Stereotactic biopsy of the right pulvinar lesion revealed glioblastoma. The tumor progressed rapidly, and the patient died 11 weeks after the onset of first symptoms.     

The clinical picture and diagnosis of optic nerve gliomas in adults

Gliomas of the optic nerve are more often in childhood and adolescence. The present paper analyzes the clinical features and diagnosis of optic nerve gliomas in 41 adult patients aged 15 to 56. Comparative analysis of the clinical picture and diagnostic specificities in optic nerve gliomas in adults and children has revealed a longer duration of the disease, less manifest exophthalmos and dilatation of the optic canal on the side of the tumor in adults. Analysis of computerized tomography data has prompted singling out 2 types of optic nerve glioma images: (1) spindle-shaped dilatation of the optic nerve in the orbit and (2) even thickening of the optic nerve along its whole length in the orbit. Each of these glioma tomographic images is associated with specific clinical features. Differential diagnosis between optic nerve gliomas and meningiomas in adults in described.     

Uveal malignant melanoma and optic nerve glioma in von Recklinghausen''s neurofibromatosis.

A case of uveal malignant melanoma and contralateral optic nerve glioma is described in a 53-year-old Caucasian male with multiple uveal melanocytic hamartomas and neurofibromatosis. The eye was enucleated, and histologically the melanoma was found to consist of 70% epithelioid cells, with many bizarre, multinucleated forms. CT scan demonstrated a non-enhancing, fusiform enlargement of the contralateral optic nerve with enlargement of the optic canal and intracranial extension. This combination of tumours has not previously been reported in a patient with neurofibromatosis and serves to emphasise the common neuroectodermal origin of tumours in this autosomal dominant condition.     

Chemotherapy for Optic Nerve Glioma in A Child with Neurofibromatosis Type-1

The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be reserved only for patients with optic chiasm or tract involvement.     

Primary tumours of the optic nerve and its sheath

PURPOSE: To describe the clinical, neuroimaging, and pathologic features of primary tumours of the optic nerve and its sheath. METHODS: Review of published cases and personal series. RESULTS: The most common primary tumour of the optic nerve is the benign glioma. This low-grade astrocytoma usually can be followed without intervention. Progression of visual symptoms and signs may necessitate either surgery to remove the tumour or radiation therapy. The most common tumour of the optic nerve sheath is the meningioma. The optimum treatment for this lesion is stereotactic or three-dimensional conformal fractionated radiation therapy, which generally results in stabilization or improvement in vision. A variety of other primary tumours may mimic, in both manifestations and imaging appearance, the more common glioma or meningioma. In such cases, the correct diagnosis may not be made until a biopsy is performed or the nerve is removed. CONCLUSION: Primary tumours of the optic nerve and its sheath are not uncommon. Diagnosis can often but not always be made by the results of a complete examination combined with imaging studies, particularly CT scanning and MR imaging. Management depends on the presumed or histologically verified nature of the tumour.     

An optic nerve tumor in von Hippel-Lindau disease, masquerading as a retinal hemangioma

CLINICAL CASE: A 35-year-old man with a family history of von Hippel-Lindau disease was diagnosed to have two retinal hemangiomas in the right eye and another in the left eye. The hemangiomas were treated with cryotherapy and laser photocoagulation respectively. Despite apparent good resolution of the retinal lesions, progressive visual loss was observed. An MRI was then performed and showed bilateral tumoral lesions of the optic nerve compatible with a glioma, meningioma or hemangioblastoma. Currently the visual acuity in his right eye is hand movement, and is 0.6 in the left eye. DISCUSSION: The presence of the retinal hemangiomas delayed the diagnosis of an optic nerve tumor in this patient.     

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.     

Optic Nerve Sheath Meningioma in the First Decade of Life: Case Report and Review of the Literature

Background/Aim

Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years. The purpose of this study is to describe an additional case and review clinical, radiologic and histopathological findings that can help differentiate such tumors in children and allow an early and accurate diagnosis.

Case Report

An 8-year-old girl had a 2-year history of progressive proptosis and total visual loss on the right side. A computed tomography scan revealed a well-defined intraconal mass with perioptic calcification. Magnetic resonance imaging showed a tumor surrounding the optic nerve and extending intracranially, with enhancement following gadolinium injection. The patient was submitted to fronto-orbital craniotomy for complete tumor excision. Histological studies identified the neoplasm as ONSM.

Conclusions

Though unusual, ONSMs may occur in children and, in these cases, present a much more aggressive behavior than in adults. ONSM should therefore be differentiated from optic glioma in children because of its aggressive behavior and need for different treatment modalities.Key Words: Optic nerve neoplasms, Benign optic nerve sheath neoplasm, Meningioma     

Pigmented malignant teratoid medulloepithelioma of ciliary body – An extremely rare intraocular tumor in adult

Malignant teratoid medulloepithelioma of ciliary body is an extremely rare tumor usually occurring in children younger than 5 years of age and is usually amelanotic. Here, we report a very rare case of pigmented malignant teratoid medulloepithelioma in a 20-year old male patient who presented initially with pain, redness and proptosis of right eyeball. The right eyeball was enucleated. A histopathological diagnosis of orbital teratoma was made and no further treatment was given. Four months later, the patient developed another swelling in the same area. A review of the previous histopathology slides revealed a pigmented malignant teratoid medulloepithelioma. No nerve involvement was seen. MRI did not show any intracranial extension. The mass along with rest of the orbital tissue was removed. No adjuvant chemotherapy or radiotherapy was given due to the lack of intracranial extension. The patient was symptom free at 2-year follow-up and the repeat MRI also showed no evidence of recurrence.     

Arachnoid cyst of the optic nerve: a case report

BACKGROUND: To report a case of an arachnoid cyst of the optic nerve with homonymous optic atrophy. HISTORY AND SIGNS: A 17-year-old female patient presented with severe visual loss, limited to light perception, of the left eye. She underwent complete ophthalmologic examination, color fundus photography and electrophysiological study. Magnetic resonance imaging (MRI) revealed the presence of a fusiform structure involving the left optic nerve from the orbital apex to the posterior surface of the globe. The clinical and radiographic features are consistent with an arachnoid cyst of the optic nerve. THERAPY AND OUTCOME: The patient refused to be treated. Two years later, the VA remained unchanged. CONCLUSIONS: The arachnoid cyst of the optic nerve is a rare entity. Its differential diagnosis from the primary optic nerve glioma is difficult and important to be made in order to be treated properly.     

Sarcoidosis of the anterior optic pathways. A diagnostic discussion

Two anatomo-clinical cases of neuro-ophthalmic sarcoidosis are reported where recognition of systemic involvement was delayed because unusual and quite isolate visual symptoms were present. The first patient had granulomas involving the intracranial optic nerve, presenting as optic nerve glioma. The second case presented as a chiasmal syndrome sharing unusual perimetric evolution. This binasal hemianopsia was probably the consequence of pre-chiasmal circulatory changes. Neuro-ophthalmic sarcoidosis should be included in the differential diagnosis of any involvement of the anterior visual pathways since these localizations are not as rare as was formerly thought. Improved awareness of this condition is a result of better neurodiagnostic studies by computed tomography which was not accurate enough a few years ago.     

Aspects on the treatment of gliomas of the anterior visual pathway

Partial excision of the glioma of the anterior visual pathway seemed to have no effect on the vision in 7 patients and had a negative effect in 2. These results support the opinion that no excision should be made in such tumours. However, the course of the disease in one exceptional patient raises doubts about the general validity of this rule. In this case the postulated chiasmal glioma proved to be an exophytic glioma arising in one intracranial optic nerve and compressing the other. Excision of the exophytic portion caused rapid and considerable improvement of vision in both eyes. Direct visualization by craniotomy was needed to separate this mode of growth from the common intrinsic growth of such tumours.     

Managing menacing meningioma

The role of irradiation therapy in the management of meningiomas involving the anterior visual system is under active clinical investigation. This report documents the value of radiotherapy in a patient with a histologically verified psammomatous tuberculum sellar meningioma and presents a 15-year postsurgical follow-up. The patient experienced a return of vision from 11/200 to 20/60 in his only seeing eye following 5,000 rads to the intracanicular and intracranial portions of right optic nerve. He is able to practice his profession 3 years following irradiation therapy. The classification of optic nerve involvement by meningioma is discussed.