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Journal of Medical Case Reports Mar 2016The coexistence of glial high grade tumors (glioblastoma, anaplastic astrocytoma) and cerebral aneurysms is common but the association with optic glioma is rare. The...
BACKGROUND
The coexistence of glial high grade tumors (glioblastoma, anaplastic astrocytoma) and cerebral aneurysms is common but the association with optic glioma is rare. The treatment of these associated lesions is problematic.
CASE PRESENTATION
A 36-year-old white woman presented to our institution with recurrent attacks of headache. Her preoperative radiological studies revealed a lesion of her left optic nerve with extension to the optic chiasma, an aneurysmatic dilatation of the left carotid bifurcation, a second aneurysm on the left of her middle cerebral artery, and a third one on her right anterior cerebral artery in the A2 tract. A left pterional approach was used to remove the tumor and clip the three aneurysmatic dilatations in a single stage.
CONCLUSIONS
We report this unusual case of optic glioma associated to multiples aneurysms and review the pertinent literature. An adequate knowledge of this association is mandatory to plan the correct approach to avoid complications.
Topics: Adult; Computed Tomography Angiography; Female; Headache Disorders; Humans; Intracranial Aneurysm; Magnetic Resonance Angiography; Optic Nerve Glioma
PubMed: 27036496
DOI: 10.1186/s13256-016-0869-8 -
Neuro-oncology Oct 2021
Topics: Benzimidazoles; Humans; Optic Nerve Glioma
PubMed: 34251024
DOI: 10.1093/neuonc/noab164 -
Brain Pathology (Zurich, Switzerland) Apr 1997Optic chiasmatic-hypothalamic gliomas (OCHGs) have been considered benign tumors and self-limiting in growth potential because of their histological appearance.... (Review)
Review
Optic chiasmatic-hypothalamic gliomas (OCHGs) have been considered benign tumors and self-limiting in growth potential because of their histological appearance. Unfortunately, most clinical series have reported significant morbidity and mortality especially with the more extensive, posteriorly positioned tumors. The biological behavior of OCHGs is age-dependent, with patients younger than five years and older than 20 years typically having tumors that exhibit aggressive growth. There are no specific pathological features to help differentiate the clinical behavior of such tumors. The emergence of modern imaging techniques, including magnetic resonance imaging (MRI), has facilitated the monitoring of the natural history of the disease and the determination of the effects of therapy. Most patients with OCHGs survive for many years. While the natural history of an OCHG for any individual may be indeterminate, enough data are now available from large series to make recommendations for treatment. Our current treatment policy for patients with OCHGs in the context of NF-I without visual failure is a conservative one involving CSF shunting for hydrocephalus if present and medical therapy for endocrinologic dysfunction. Patients with or without NF-I with visual deterioration or progressive neurological deficits and a rapidly expanding suprasellar mass lesion are treated surgically. After tumor resection, patients whose vision is significantly compromised or who show progression of their disease on serial neuroimaging scans receive chemotherapy. If chemotherapy proves ineffective in disease stabilization, then considerations of radiation therapy are given to children over five years old.
Topics: Child, Preschool; Cranial Nerve Neoplasms; Glioma; Humans; Hypothalamic Neoplasms; Magnetic Resonance Imaging; Optic Chiasm
PubMed: 9161730
DOI: 10.1111/j.1750-3639.1997.tb01065.x -
Arquivos de Neuro-psiquiatria Jun 2015Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and...
Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.
Topics: Disease Management; Humans; Neurilemmoma; Neurofibromatoses; Neurofibromatosis 1; Neurofibromatosis 2; Optic Nerve Glioma; Risk Factors; Skin Neoplasms
PubMed: 26083891
DOI: 10.1590/0004-282X20150042 -
Annual Review of Pathology 2012Neurofibromatosis type 1 (NF1) is a genetic disease that results from either heritable or spontaneous autosomal dominant mutations in the NF1 gene. A second-hit mutation... (Review)
Review
Neurofibromatosis type 1 (NF1) is a genetic disease that results from either heritable or spontaneous autosomal dominant mutations in the NF1 gene. A second-hit mutation precedes the predominant NF1 neoplasms, which include myeloid leukemia, optic glioma, and plexiform neurofibroma. Despite this requisite NF1 loss of heterozygosity in the tumor cell of origin, nontumorigenic cells contribute to both generalized and specific disease manifestations. In mouse models of plexiform neurofibroma formation, Nf1 haploinsufficient mast cells promote inflammation, accelerating tumor formation and growth. These recruited mast cells, hematopoietic effector cells long known to permeate neurofibroma tissue, mediate key mitogenic signals that contribute to vascular ingrowth, collagen deposition, and tumor growth. Thus, the plexiform neurofibroma microenvironment involves a tumor/stromal interaction with the hematopoietic system that depends, at the molecular level, on a stem cell factor/c-kit-mediated signaling axis. These observations parallel findings in other NF1 disease manifestations and are clearly relevant to medical management of these neurofibromas.
Topics: Animals; Antineoplastic Agents; Benzamides; Hematopoietic System; Humans; Imatinib Mesylate; Mast Cells; Neurofibroma, Plexiform; Neurofibromatosis 1; Neurofibromin 1; Piperazines; Proto-Oncogene Proteins c-kit; Pyrimidines; Signal Transduction; Stem Cell Factor; Tumor Microenvironment
PubMed: 22077553
DOI: 10.1146/annurev-pathol-011811-132441 -
The Oncologist 2000Neurofibromatosis type 1 (NF1) represents a major risk factor for development of malignancy, particularly malignant peripheral nerve sheath tumors (MPNST), optic... (Review)
Review
Neurofibromatosis type 1 (NF1) represents a major risk factor for development of malignancy, particularly malignant peripheral nerve sheath tumors (MPNST), optic gliomas, other gliomas, and leukemias. The oncologist will see NF1 patients referred for treatment of malignancy, and should be alert to the possibility of undiagnosed NF1 among patients with cancer. Brain tumors tend to have a more indolent course in NF1 than in the general population, and hence are best managed conservatively. MPNST, in contrast, do not respond to standard chemotherapy or radiation therapy. The most effective treatment of MPNST appears to be early diagnosis and surgery, but early diagnosis is hampered by frequent occurrence within preexisting large tumors, making new growth or change difficult to detect. New insights into pathogenesis now offer hope of development of specific methods of treatment with reduced toxicity and more precise molecular targeting. There is an urgent need, however, to develop methods to measure tumor growth and monitor outcomes, develop preclinical drug screening systems, and further explore the pathogenesis of the disorder to determine whether mechanisms other than Ras regulation may be important in pathogenesis.
Topics: Glioma; Humans; Leukemia; Neurilemmoma; Neurofibromatosis 1; Phenotype
PubMed: 11110599
DOI: 10.1634/theoncologist.5-6-477 -
NeuroImage. Clinical 2020The subventricular zone of the third ventricle (TVZ) is a germinal stem cell niche, identified as the possible location of optic pathway glioma (OPG) cell origin....
BACKGROUND
The subventricular zone of the third ventricle (TVZ) is a germinal stem cell niche, identified as the possible location of optic pathway glioma (OPG) cell origin. Paediatric OPGs are predominantly diagnosed as low-grade astrocytomas, which are either sporadic or are associated with neurofibromatosis type-1 (NF1). These tumours often cause a significant impairment to visual acuity (VA). Infiltrative/invasive tumour activity is associated with increased apparent diffusion coefficient (ADC) and cerebral blood flow (CBF). This study aimed to determine whether TVZ imaging features differed between sporadic-OPG, NF1-OPG and controls, and whether the ADC and CBF profile at the germinal stem cell niche (the TVZ) correlated with the primary outcome of VA.
METHODS
ADC and CBF MRI data were acquired from 30 paediatric OPG patients (median age 6 years; range 8 months-17 years), along with VA measurements, during clinical surveillance of their tumour. Values for mean ADC and maximum CBF were measured at the TVZ, and normalized to normal-appearing grey matter. These values were compared between the two OPG groups and the healthy control subjects, and multivariate linear regression was used to test the linear association between these values and patient's VA.
RESULTS
In the TVZ, normalized mean ADC was higher in NF1-associated OPG patients (N = 15), compared to both sporadic OPG patients (N = 15; p = 0.010) and healthy controls (N = 14; p < 0.001). In the same region, normalized maximum CBF was higher in sporadic OPG patients compared to both NF1-OPG patients (p = 0.016) and healthy controls (p < 0.001). In sporadic OPG patients only, normalized mean ADC in the TVZ was significantly correlated with visual acuity (R = 0.41, p = 0.019). No significant correlations were found between TVZ CBF and ADC values and visual acuity in the NF1-associated OPG patients.
CONCLUSION
Quantitative MRI detects TVZ abnormalities in both sporadic and NF1-OPG patients, and identifies TVZ features that differentiate the two. TVZ features may be useful MRI markers of interest in future predictive studies involving sporadic OPG.
Topics: Child; Humans; Infant; Lateral Ventricles; Magnetic Resonance Imaging; Neurofibromatosis 1; Optic Nerve Glioma; Third Ventricle
PubMed: 33038669
DOI: 10.1016/j.nicl.2020.102447 -
NeuroImage. Clinical 2018Radiological biomarkers which correlate with visual function are needed to improve the clinical management of optic pathway glioma (OPG) patients. Currently, these are...
BACKGROUND
Radiological biomarkers which correlate with visual function are needed to improve the clinical management of optic pathway glioma (OPG) patients. Currently, these are not available using conventional magnetic resonance imaging (MRI) sequences. The aim of this study was to determine whether diffusion MRI could be used to delineate the entire optic pathway in OPG patients, and provide imaging biomarkers within this pathway which correlate with a patient's visual acuity (VA).
METHODS
Multi-shell diffusion MRI data were acquired in a cohort of paediatric OPG patients, along with VA measurements in each eye. Diffusion MRI data were processed using constrained spherical deconvolution and probabilistic fibre tractography, to delineate the white matter bundles forming the optic pathway in each patient. Median fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were measured in the optic nerves, tracts, and radiations, and correlated against each patient's VA.
RESULTS
In the optic nerves, median FA significantly correlated with VA (R = 0.31, = 0.0082), with lower FA associated with poorer vision. In the optic radiations, both lower FA and higher ADC were significantly associated with poorer vision (R = 0.52, = 0.00075 and R = 0.50, = 0.0012 respectively). No significant correlations between VA and either FA or ADC were found in the optic tracts.
CONCLUSIONS
Multi-shell diffusion MRI provides delineation of the optic pathway in OPG patients, despite the presence of tumour invasion. This technique provides imaging biomarkers which are sensitive to microstructural damage to the underlying white matter in this pathway, which is not always visible on conventional MRI.
Topics: Adolescent; Anisotropy; Brain Neoplasms; Child; Child, Preschool; Cohort Studies; Diffusion Tensor Imaging; Female; Glioma; Humans; Image Processing, Computer-Assisted; Infant; Linear Models; Male; Neurofibromatosis 1; Optic Nerve; Visual Acuity; Visual Pathways
PubMed: 29527480
DOI: 10.1016/j.nicl.2017.10.010 -
Journal of Clinical Imaging Science 2021Vision loss can occur due to a variety of etiologies along the primary visual pathway. Understanding the anatomic organization of the visual pathway, which spans the... (Review)
Review
Vision loss can occur due to a variety of etiologies along the primary visual pathway. Understanding the anatomic organization of the visual pathway, which spans the globe to the occipital cortex, can help tailor neuroimaging to identify the cause of visual dysfunction. In this review, relevant anatomy and optimization of computed tomography and magnetic resonance imaging techniques will be described. This will be followed by a discussion of imaging findings related to pathologies at each functional anatomic level.
PubMed: 33880244
DOI: 10.25259/JCIS_12_2021 -
The British Journal of Ophthalmology Dec 1989Thirty-one patients presenting as orbital optic nerve glioma have been reviewed with maximum follow-up of 14 years. Sixteen of these patients have been reported on...
Thirty-one patients presenting as orbital optic nerve glioma have been reviewed with maximum follow-up of 14 years. Sixteen of these patients have been reported on previously and further follow-up is provided. Sixteen patients had a stable clinical course with little change over a period of up to 13.5 years. Neurofibromatosis was relatively common in this group (11/16). Fifteen patients had progressive enlargement of the tumour; the incidence of neurofibromatosis in this group was low (4/15). Eleven of these patients were explored neurosurgically and the optic nerve totally excised in 10 of them. The proximal cut end was normal in six patients and the chiasm has apparently remained free of tumour in all of them. We suggest a method of management of primary optic nerve tumours, both meningiomas and gliomas, in young patients.
Topics: Adolescent; Adult; Child; Child, Preschool; Cranial Nerve Neoplasms; Female; Follow-Up Studies; Glioma; Humans; Male; Optic Nerve Diseases
PubMed: 2611193
DOI: 10.1136/bjo.73.12.967