scholarly journals Neuroophthalmological management of optic pathway gliomas

2007 ◽  
Vol 23 (5) ◽  
pp. E1 ◽  
Author(s):  
Andrew G. Lee
Keyword(s):  
Growth Rate ◽  
Optic Nerve ◽  
Major Complication ◽  
Initial Step ◽  
Neurofibromatosis Type ◽  
Tumor Location ◽  
Optic Pathway ◽  
First Line ◽  
Younger Patients ◽  
Optic Pathway Gliomas

✓ The growth rate of optic pathway gliomas (OPGs) is unpredictable and quite variable, especially in children with neurofibromatosis Type 1 (NF1). Close neuroophthalmalogical clinical follow-up with serial imaging (magnetic resonance imaging of the brain with and without contrast enhancement) is the recommended initial step in management to establish the growth rate of the lesion in an individual patient. Typically, only symptomatic and/or radiographically growing tumors require treatment, and observation is the accepted first-line option. Although both chemotherapy and radiotherapy can stabilize growth or even decrease the size of tumors, chemotherapy, especially in younger patients, has fewer side effects than radiation therapy (such as secondary tumors, radiation necrosis, and Moyomoya disease) and is generally considered the first-line treatment for progressive lesions in younger patients. The tumor location defines prognosis in OPGs; optic nerve gliomas (ONG) have the lowest rate of complications and death, and optic chiasm and retrochiasmal gliomas the highest. Although the major complication of an OPG is visual loss, hypothalamic involvement can lead to death. Resection is an option for ONGs but is generally reserved for tumors confined to the optic nerve with poor or no vision, or for patients with severe, cosmetically unappealing proptosis, producing severe pain or exposure keratopathy in a blind eye. Resection is generally not an option for intrinsic chiasmal or retrochiasmal OPGs. Extrinsic (exophytic) components can be debulked surgically, and surgery can be performed for hydrocephalus (ventriculoperitoneal shunt placement). The approach to a patient with OPG must be individualized based on tumor location, radiographic or clinical progression, the presence of NF1, and a risk–benefit comparison for treatment.

Optic pathway gliomas: a review

2007 ◽  
Vol 23 (5) ◽  
pp. E2 ◽  
Author(s):  
Mandy J. Binning ◽  
James K. Liu ◽  
John R. W. Kestle ◽  
Douglas L. Brockmeyer ◽  
Marion L. Walker
Keyword(s):  
Progressive Disease ◽  
Clinical Course ◽  
Mass Effect ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Optic Pathway ◽  
Intracranial Tumors ◽  
First Line ◽  
Optic Pathway Gliomas

✓Optic pathway gliomas represent approximately 3–5% of childhood intracranial tumors. They usually occur in children during the first decade of life and are seen in 11–30% of patients with neurofibromatosis Type 1 (NF1). Although these tumors are typically low-grade gliomas, the clinical course and natural history are highly variable, making treatment paradigms difficult. Overall, however, they are often indolent tumors that can be observed over time for progression without initial treatment, especially in patients with NF1. Chemotherapy is the first-line treatment for progressive tumors, and radiation therapy is reserved for patients with progressive disease who are older than 5–7 years. Surgery is reserved for large tumors causing mass effect or hydrocephalus and tumors confined to the orbit or unilateral optic nerve.

scholarly journals LGG-14. PRESENTATION CHARACTERISTICS AND TREATMENT OUTCOMES FOR PEDIATRIC OPTIC PATHWAY GLIOMAS IN QATAR

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i34-i34
Author(s):  
Ata Maaz ◽  
Tayseer Yousif ◽  
Mohammed Abdulmajeed ◽  
Moegamad Ederies ◽  
Pedro Neri ◽  
...  
Keyword(s):  
Visual Impairment ◽  
Treatment Outcomes ◽  
Progression Free Survival ◽  
Neurofibromatosis Type ◽  
Visual Assessment ◽  
Optic Pathway ◽  
First Line ◽  
Visual Outcomes ◽  
First Line Therapy ◽  
Optic Pathway Gliomas

Abstract Objectives To review the presentation characteristics and treatment outcomes for pediatric optic pathway gliomas (OPG) in Qatar. Methods Retrospective review of data for children with OPG from January 2009 to February 2021. Presenting features, diagnostic imaging and indications for treatment were reviewed. Progression free survival (PFS) and overall survival(OS) were computed using standard statistical methods. Medical notes were also reviewed for visual outcomes. Results Nineteen patients were diagnosed with OPG during the study period. There were 10 (52%) females. Median age was 29 months (range 6–186) months. Eleven (57%) tumors were related to neurofibromatosis Type 1 (NF-1). Nine (47%) of OPG were located in optic nerves, 5 (26%) were chiasmatic/suprasellar, while the remaining 5(26%) involved a combination of structures. Seven(36%) children presented with oculo-visual symptoms. Another 7 were diagnosed on screening imaging for NF-1. Seven(36%) children had debulking surgery/biopsy, while the remaining patients were diagnosed on neuro-imaging alone. Thirteen (68%) patients were treated with chemotherapy and 2 received additional radiotherapy. Indications for non-surgical treatment included visual impairment (46%) and large/progressive tumor (54%). Carboplatin based regimes were used as first line chemotherapy for 76 % of patients. Five (38%) patients required more than one lines of treatment. OS and PFS at 36 months were 100% and 48%. Baseline visual assessment showed 5 children each (26%) had unilateral and bilateral visual impairment, while 9 (48%) had normal vision. Of the 6 children receiving chemotherapy for visual impairment, 2 (33%) showed improvement. Of the 7 children treated for large/progressive tumors, 3 (42%) showed partial response, 2(28%) had progressive disease and 1 had stable disease after the first line therapy. Conclusions Our results are in-keeping with international data for optic pathway gliomas. Early referral and diagnosis may improve visual outcomes for this group of tumors.

Outcome of neurofibromatosis type 1 patients treated with first line vinblastine for optic pathway gliomas: A Canadian multicenter study.

2015 ◽  
Vol 33 (15_suppl) ◽  
pp. 2019-2019 ◽  
Author(s):  
Alvaro Lassaletta ◽  
Katrin Scheinemann ◽  
Shayna M. Zelcer ◽  
Juliette Hukin ◽  
Beverley Wilson ◽  
...  
Keyword(s):  
Multicenter Study ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway ◽  
First Line ◽  
Optic Pathway Gliomas

Follow-Up of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1

1994 ◽  
Vol 25 (06) ◽  
pp. 295-300 ◽  
Author(s):  
Ch. Kuenzle ◽  
M. Weissert ◽  
E. Roulet ◽  
H. Bode ◽  
S. Schefer ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway ◽  
Optic Pathway Gliomas

scholarly journals Syndromic and sporadic pediatric optic pathway gliomas: review of clinical and histopathological differences and treatment implications

2007 ◽  
Vol 23 (5) ◽  
pp. E3 ◽  
Author(s):  
Mohammed F. Shamji ◽  
Brien G. Benoit
Keyword(s):  
Management Strategies ◽  
Neurofibromatosis Type ◽  
Endocrine Function ◽  
Physical Growth ◽  
Secondary Malignancy ◽  
Optic Pathway ◽  
Oncogenic Signaling ◽  
Treatment Protocols ◽  
Molecular Abnormalities ◽  
Optic Pathway Gliomas

✓Optic pathway gliomas (OPGs) are the most common primary neoplasm of the optic pathway. These lesions usually present in childhood and can arise anywhere along the optic pathway; they occur more frequently in women; and they rarely undergo late progression. Management strategies after the initial diagnosis are controversial, compounded by the different behaviors exhibited by sporadic and syndromic tumors. Neurofibromatosis Type 1 (NF1), with aberrant oncogenic signaling and consequent predisposition to intracranial tumors, is the most common associated syndrome, with nearly 20% of NF1 patients developing OPGs. A comorbid NF1 diagnosis has implications for tumor location with greater predilection for optic nerve involvement, whereas chiasmal and postchiasmal lesions are more frequently seen in sporadic cases. Syndromic OPGs often exhibit more indolent behavior and lower rates of clinical progression, and the majority of these are diagnosed by routine neuroophthalmological screening. When treatment is indicated, however, the molecular abnormalities that constitute this syndrome can limit the available chemotherapy and radiotherapy options because clinicians fear secondary malignancy and cerebrovascular complications. Furthermore, radiotherapy early in life can impair an individual's intellectual development, endocrine function, and physical growth, thereby limiting the role of this modality in the treatment of this childhood lesion. Differential gene expression and histogenesis among sporadic and syndromic OPGs may account for the different tumor behaviors, but studies correlating specific genetic and proteomic changes with patient outcome are pending. Loss of heterozygosity at 10 and 17q are more common among patients with NF1, and Ki67 labeling intensity of 2–3% and low p53 labeling intensity seem prognostic of aggressive tumor behavior. Recent advances in the development of a preclinical mouse model of NF1-associated OPG will permit investigation into improved detection strategies and chemotherapeutic and radiotherapy treatment protocols.

scholarly journals Optic pathway gliomas associated with neurofibromatosis type I in children

2019 ◽  
Vol 18 (4) ◽  
pp. 29-38
Author(s):  
E. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
O. A. Medvedeva ◽  
Y. Y. Trunin ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Survival Rate ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
National Medical ◽  
Optic Pathway Gliomas ◽  
Visual Disturbances

Neurofibromatosis type I (NFI) is one of the most common brain tumor predisposition syndromes. Children with NFI are prone to develop a low grade gliomas, which can be localized in various areas of the brain, however, most of them occur in the structures of the optic pathway: optic nerves, chiasm, tracts and optic radiations – that is, are optic pathway gliomas (OPG). This retrospective study included children with newly diagnosied low grade glioma of the optic pathway at the age from 0 to 18 years with NFI, who underwent medical examination and / or treatment at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31,2015. Atotal from 264 patients 42 (16%) had clinical manifestations of NFI. The ratio of boys and girls was 1:1. The median age was 4.25 years (range 4.5 months – 17 years). Visual disturbances were the most frequent clinical manifestation of the tumor. Surgical resection was performed in 18 patients. The remaining 24 patients OPG were diagnosed based on clinical and radiological findings: 9 patients were in observation group, 11 patients chemotherapy was carried out, three were given radiation therapy, and spontaneous regression of the tumor was recorded in 1 patient. Progression of the disease was observed in 14 patients in our cohort. The overall survival rate in patients with NFI was 98 ± 2% at 5 years. Event free survival rate was 68 ± 7% at 5 years.The study was approved by the Independent Ethics Committee of N.N. Burdenko National Medical Research Center of neurosurgery Ministry of healthcare ofRussian Federation.

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