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Cancer Cell Aug 2021In a recent issue of Nature, Pan and colleagues explore whether light-induced stimulation of the optic nerve could influence the biology of optic gliomas. This study...
In a recent issue of Nature, Pan and colleagues explore whether light-induced stimulation of the optic nerve could influence the biology of optic gliomas. This study highlights the importance of neuronal-glioma interactions.
Topics: Humans; Neurons; Optic Nerve; Optic Nerve Glioma; Tumor Microenvironment
PubMed: 34375609
DOI: 10.1016/j.ccell.2021.07.010 -
Child's Nervous System : ChNS :... Oct 2020Optic Pathway Gliomas (OPG) are the most common brain tumor in Neurofibromatosis 1 patients (NF1). They are found along the optic pathway and may involve the optic...
BACKGROUND
Optic Pathway Gliomas (OPG) are the most common brain tumor in Neurofibromatosis 1 patients (NF1). They are found along the optic pathway and may involve the optic nerves, chiasm, retro-chiasmatic structures, and the optic radiations. NF1 associate OPG (NF1-OPG) have variable presentation, disease course and response to treatment. The optimal management is patient-specific and should be tailored by a multidisciplinary team. Age, sex, histology, and molecular markers may be important factors in the individualized decision-making process. Chemotherapy is the first-line treatment in cases of progressive tumors, and visual preservation is the main goal of treatment.
PURPOSE
In this paper we will review the disease, practical management, and recent advances of NF1-OPG.
Topics: Eye; Humans; Nerve Fibers; Neurofibromatosis 1; Optic Nerve; Optic Nerve Glioma; Optic Nerve Neoplasms
PubMed: 32524182
DOI: 10.1007/s00381-020-04697-1 -
Eye (London, England) Oct 2006To describe cases of optic pathway glioma (OPG) warranting treatment in children.
PURPOSE
To describe cases of optic pathway glioma (OPG) warranting treatment in children.
METHODS
This is a retrospective review of pediatric patients treated for OPG. The clinical data and imaging studies were obtained from the medical records and radiology files of patients seen at the Pediatric Neuro-Ophthalmology Clinic at the University of Illinois, Chicago and the private office of the author (LMK).
RESULTS
A total of seven cases with an age range of 3-48 months at presentation were reviewed. Three of the patients were also ultimately diagnosed with neurofibromatosis type 1. Presenting symptoms included proptosis, decreased vision, gaze deficit, and nystagmus. Four patients underwent biopsies that confirmed OPG. Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle. One patient was successfully treated with an en-bloc optic nerve excision. Two patients underwent unilateral enucleation owing to globe complications.
CONCLUSION
Although benign tumours, OPG can behave very aggressively in young children. Both chemotherapy and en-bloc excision can be employed for treatment.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child, Preschool; Disease Progression; Exophthalmos; Eye Enucleation; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Neoplasm Invasiveness; Neurofibromatosis 1; Optic Nerve; Optic Nerve Glioma; Retrospective Studies; Treatment Outcome; Vision Disorders
PubMed: 17019413
DOI: 10.1038/sj.eye.6702379 -
Radiologie (Heidelberg, Germany) Mar 2024Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can... (Review)
Review
Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can be initiated promptly. Neuroradiological imaging, in particular magnetic resonance imaging (MRI), plays an important role in the diagnostic of orbital tumours. In adults, lymphoproliferative diseases, inflammations and secondary orbital tumours are most frequently found, whereas in children mostly dermoid cysts, optic gliomas and capillary haemangiomas are found. Optic glioma is a pilocytic astrocytoma and accounts for two thirds of all primary optic tumours. Optic nerve sheath meningiomas mostly affect middle-aged women. In childhood, retinoblastoma is the most common intraocular tumour. This is an aggressive malignant tumour which can occur unilaterally or bilaterally. Based on the imaging findings, differential diagnoses can usually be easily narrowed down using criteria such as age of manifestation, frequency, localisation and imaging characteristics.
Topics: Adult; Child; Middle Aged; Humans; Female; Orbital Neoplasms; Orbital Diseases; Optic Nerve Neoplasms; Optic Nerve Glioma; Meningeal Neoplasms; Retinal Neoplasms
PubMed: 38194103
DOI: 10.1007/s00117-023-01257-x -
Neurology Dec 2016To determine whether tumor size is associated with retinal nerve fiber layer (RNFL) thickness, a measure of axonal degeneration and an established biomarker of visual...
OBJECTIVE
To determine whether tumor size is associated with retinal nerve fiber layer (RNFL) thickness, a measure of axonal degeneration and an established biomarker of visual impairment in children with optic pathway gliomas (OPGs) secondary to neurofibromatosis type 1 (NF1).
METHODS
Children with NF1-OPGs involving the optic nerve (extension into the chiasm and tracts permitted) who underwent both volumetric MRI analysis and optical coherence tomography (OCT) within 2 weeks of each other were included. Volumetric measurement of the entire anterior visual pathway (AVP; optic nerve, chiasm, and tract) was performed using high-resolution T1-weighted MRI. OCT measured the average RNFL thickness around the optic nerve. Linear regression models evaluated the relationship between RNFL thickness and AVP dimensions and volume.
RESULTS
Thirty-eight participants contributed 55 study eyes. The mean age was 5.78 years. Twenty-two participants (58%) were female. RNFL thickness had a significant negative relationship to total AVP volume and total brain volume (p < 0.05, all comparisons). For every 1 mL increase in AVP volume, RNFL thickness declined by approximately 5 microns. A greater AVP volume of OPGs involving the optic nerve and chiasm, but not the tracts, was independently associated with a lower RNFL thickness (p < 0.05). All participants with an optic chiasm volume >1.3 mL demonstrated axonal damage (i.e., RNFL thickness <80 microns).
CONCLUSIONS
Greater OPG and AVP volume predicts axonal degeneration, a biomarker of vision loss, in children with NF1-OPGs. MRI volumetric measures may help stratify the risk of visual loss from NF1-OPGs.
Topics: Adolescent; Axons; Child; Child, Preschool; Cross-Sectional Studies; Female; Humans; Magnetic Resonance Imaging; Male; Neurofibromatosis 1; Optic Nerve; Optic Nerve Glioma; Organ Size; Retinal Degeneration; Tomography, Optical Coherence; Tumor Burden; Visual Pathways
PubMed: 27815398
DOI: 10.1212/WNL.0000000000003402 -
World Neurosurgery Sep 2019Optic pathway gliomas and glioblastomas remain a rare entity within the infant population.
BACKGROUND
Optic pathway gliomas and glioblastomas remain a rare entity within the infant population.
CASE DESCRIPTION
We outline the case of a 6-month-old female who presented with failure to thrive, nystagmus and features of raised intracranial pressure. Subsequent magnetic resonance imaging demonstrated an infiltrating tumor radiating from the optic nerves bilaterally. She underwent emergent ventriculoperitoneal shunting and biopsy. Histology confirmed a World Health Organization grade IV glioblastoma.
CONCLUSIONS
The patient remained clinically and radiologically stable at 1 year. Optic pathway glioblastoma in this population is a previously undescribed entity that requires multidisciplinary input to guide ongoing therapy.
Topics: Brain Neoplasms; Female; Glioblastoma; Humans; Infant; Optic Nerve Glioma
PubMed: 31158532
DOI: 10.1016/j.wneu.2019.05.236 -
Zhurnal Voprosy Neirokhirurgii Imeni N.... 2016The article presents two rare clinical cases of low-grade (WHO grade I-II) glioma of the anterior visual pathway structures, the chiasm and optic nerves, in adults. The... (Review)
Review
The article presents two rare clinical cases of low-grade (WHO grade I-II) glioma of the anterior visual pathway structures, the chiasm and optic nerves, in adults. The feature of these cases was the benign nature of a chiasm and optic nerve glioma in adults as well as its presentation in the form of hemorrhage to the tumor and parenchymal and subarachnoid space, which to some extent complicated making the correct diagnosis. Removal of an intracerebral hematoma and open tumor biopsy were performed in one case, and removal of a hematoma and partial tumor resection followed by radiotherapy were performed in the other case.
Topics: Adolescent; Adult; Female; Humans; Magnetic Resonance Imaging; Optic Chiasm; Optic Nerve Glioma; Subarachnoid Hemorrhage
PubMed: 28635693
DOI: 10.17116/neiro201680590-97 -
Pediatric Blood & Cancer May 2006Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a... (Review)
Review
Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children and infants further complicates selection of therapy. Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice. Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically. An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy. Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.
Topics: Combined Modality Therapy; Cranial Nerve Neoplasms; Humans; Optic Nerve Glioma; Treatment Outcome
PubMed: 16411210
DOI: 10.1002/pbc.20655 -
Neurosurgical Review Jan 2017Optic pathway glioma (OPG) encompasses a spectrum of findings ranging from lesions confined to the optic nerve only, lesions affecting the optic chiasm and hypothalamus,...
Optic pathway glioma (OPG) encompasses a spectrum of findings ranging from lesions confined to the optic nerve only, lesions affecting the optic chiasm and hypothalamus, and lesions with diffuse involvement of a large part of the optic pathway and neighboring structures. The majority of pediatric low-grade astrocytomas in the optic/chiasmatic region are typical pilocytic astrocytoma. The rest of them (10 %) may be other gliomas such as fibrillary pilomyxoid astrocytoma (grade 2 WHO). The postsurgical local recurrence rate of 55 to 76 % has been reported in some histological subtypes such as pilomyxoid astrocytoma (grade 2). Performing a prechiasmatic transection might offer a new surgical option to avoid further tumor growth toward the chiasm in the optic nerve glioma with predominantly orbital manifestations. In this retrospective study, four patients (three children, two without neurofibromatosis type 1 (NF1), and one with NF1 and one adult without NF1) with optic nerve glioma without involvement of the chiasm but blindness, disfiguring proptosis, and pain of the affected eye were included. The surgical approach was performed as a combined approach from pterional extradural and intradural. Without any exceptions, vision of the contralateral eye could be preserved and did not show any deterioration after surgery or during the follow-up time between 17 and 106 months. Furthermore, in all patients, gross total tumor resection could be achieved. During follow-up observation in all patients, no further tumor progress or recurrences could be observed. None of the patients were treated postoperatively by radiotherapy or chemotherapy. Prechiasmatic transection of the optic nerve in optic nerve glioma without affecting the chiasm might offer a surgical treatment option to control tumor growth and to preserve vision of the contralateral eye.
Topics: Child; Child, Preschool; Female; Humans; Hypothalamus; Infant; Magnetic Resonance Imaging; Male; Neoplasm Recurrence, Local; Neurosurgical Procedures; Optic Nerve; Optic Nerve Glioma; Optic Nerve Neoplasms; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 27230830
DOI: 10.1007/s10143-016-0747-2 -
Neuro-Chirurgie Mar 2018Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the... (Review)
Review
INTRODUCTION
Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the internal carotid arteries, which induces the development of abnormal neovessels in the deep regions of the brain. Some pathologies such as sickle cell disease, Down syndrome or Graves' disease may be associated with Moya-Moya angiopathy. These syndromic forms harbor several differences compared with idiopathic Moya-Moya disease.
CASE REPORT
We report the case of a young patient who presented with a syndromic form of Moya-Moya angiopathy after cranial radiation therapy for an optic glioma associated with type 1 neurofibromatosis treated by combined revascularization. We discuss the particularities of syndromic forms, in their presentation and management based on a review of the literature.
CONCLUSION
Many diseases can be associated with Moya-Moya syndrome. Symptomatic patients should undergo surgery, but the risk of postoperative complications appears to be greater than that encountered in patients with non-syndromic Moya-Moya angiopathy.
Topics: Cerebral Hemorrhage; Cerebral Revascularization; Child, Preschool; Cranial Irradiation; Female; Humans; Moyamoya Disease; Neurofibromatosis 1; Optic Nerve Glioma; Syndrome; Young Adult
PubMed: 29475609
DOI: 10.1016/j.neuchi.2017.11.004