Postoperative hydrocephalus is a high-risk lethal factor for patients with low-grade optic pathway glioma

2021 ◽  
pp. 1-7
Author(s):  
Song Han ◽  
Zuocheng Yang ◽  
Liguo Wang ◽  
Yakun Yang ◽  
Xueling Qi ◽  
...  
Keyword(s):  
High Risk ◽  
Lethal Factor ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway ◽  
Postoperative Hydrocephalus

scholarly journals Single-Agent Oral Vinorelbine in the Treatment of Pediatric Progressive Optic Pathway Glioma: A Single Institutional Experience

2021 ◽  
pp. 1-6
Author(s):  
Natalia Dassi ◽  
Natalia Dassi ◽  
N.S. Silva ◽  
F.A. Silva ◽  
D.B. Almeida ◽  
...  
Keyword(s):  
Single Agent ◽  
Drug Acquisition ◽  
Gastrointestinal Toxicity ◽  
Optic Pathway Glioma ◽  
Oral Drug ◽  
Low Grade ◽  
Optic Pathway ◽  
Oral Vinorelbine ◽  
Minor Response ◽  
Response To Chemotherapy

Purpose: The vinca alkaloids’ activity against pediatric low-grade glioma (PLGG) is well established. The goal of the present study is to describe our experience with oral vinorelbine in patients with progressive optic pathway glioma (OPG), not only regarding the clinical response, but also the cost benefit using an oral medication. Methods: Patients under 21 years of age with unresectable and/or progressive OPG were eligible. Oral vinorelbine was administered at a dose of 90mg/m2 daily on days 0, 8 and 22, in a scheme of 4 weekly cycles for a total of 18 cycles (54 doses). Results: From 2013 to 2018, sixteen patients were enrolled onto the study, with a median age of 9,1 years (range 4,6-17,8y). The most common histology was pilocytic astrocytoma (88,8%). Best response to chemotherapy was reviewed with a response rate (complete, partial, or minor response) of 30% for the patients treated exclusively with the oral drug. Five-year event-free survival (EFS) rate was 43.4%. Six patients had to change to intravenous vinorelbine due to gastrointestinal toxicity, vomiting grade III. None of the patients showed neurotoxicity. The total cost including drug acquisition, administration and toxicity management was lower with the oral formulation comparing to IV one. Conclusion: Single-agent oral vinorelbine seems to have some clinical activity in the management of recurrent or refractory pediatric OPG, being an interesting and cost-effective option, minding that gastrointestinal toxicity may be limiting and a combination of antiemetics should be considered in this treatment regimen.

scholarly journals RONC-05. PRESERVING VISION IN OPTIC PATHWAY GLIOMA AMONG PATIENTS WITHOUT NEUROFIBROMATOSIS TYPE 1

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii457-iii457
Author(s):  
Alexander Hanania ◽  
Arnold Paulino ◽  
Ethan Ludmir ◽  
Veeral Shah ◽  
Susan McGovern ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Initial Therapy ◽  
Optic Pathway Glioma ◽  
Rank Test ◽  
Low Grade ◽  
Optic Pathway ◽  
Longitudinal Assessment ◽  
Local Progression ◽  
Age Appropriate ◽  
Modern Era

Abstract PURPOSE Sporadic optic pathway/hypothalamic gliomas (OP/HGs) represent a unique entity within pediatric low-grade glioma. Despite favorable survival, the location makes treatment difficult and local progression debilitating. We conducted longitudinal assessment of visual acuity (VA) among patients treated in the modern era with chemotherapy (CT) or early radiotherapy (RT). METHODS Clinical characteristics were abstracted for patients treated over a 15-year period (2000–2015) at a single institution. Comprehensive ophthalmologic data taken at three to six-month intervals was examined with age-appropriate VA metrics converted to LogMAR scale. Kaplan-Meir “blindness-free survival” (BFS) curves were calculated as time to bilateral functional blindness (i.e. LogMAR ≥ 0.8 in both eyes), stratified by treatment and compared using log-rank test. RESULTS Thirty-six patients with median follow-up of 7.6 years (range: 2–17) were identified. Median age at diagnosis was 2.5 years (IQR: <1–5). Early RT was administered as initial therapy (n=6) or first-line salvage (n=5) in a total of eleven patients (31%) at a mean age of 12 years (range: 6–17). Twenty-five patients (69%) were maintained primarily on CT with a mean age at initiation of 2.4 years (range <1–8). Of these, five patients received RT after ≥2 systemic therapy regimens. In terms of visual preservation, five/eight-year BFS rates were 84%/59% and 100%/100%, for CT and early RT, respectively (p=0.046). CONCLUSIONS In a contemporary cohort, early RT, defined as initial or 1st line salvage therapy for OP/HGs manifested in superior VA. Children undergoing CT are at highest risk of functional blindness following five years of treatment.

scholarly journals PATH-30. TARGETED THERAPIES FOR PEDIATRIC LOW-GRADE GLIOMAS: A CASE SERIES

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi149-vi149
Author(s):  
Nidhi Shah ◽  
Andrew Walter ◽  
Gurcharanjeet Kaur
Keyword(s):  
Targeted Therapies ◽  
Case Series ◽  
Braf V600e ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway ◽  
Intravenous Chemotherapy ◽  
Low Grade Gliomas ◽  
Patient Reported ◽  
Cns Malignancies

Abstract Pediatric central nervous system (CNS) malignancies are the most common malignancies of childhood. The standard treatment plan for most CNS malignancies involves surgery, chemotherapy, radiation, and/or a combination of above therapies. Unresectable symptomatic low grade gliomas, such as pilocytic astrocytomas and gangliogliomas, are slow growing tumors that are typically responsive to a single course of intravenous chemotherapy, but in select patients these WHO grade I or II tumors can recur and be refractory to multiple courses. Molecular diagnostics can offer valuable insight into the tumor microenvironment, where targeted therapies can be offered for specific actionable mutations. Here we report a case series of 3 pediatric patients, with unique CNS malignancies, currently on targeted therapies for tumor-specific somatic mutations. Patient A is a 5 year old male with unresectable Neurofibromatosis-1 related plexiform neurofibroma of the nasopharyngeal space as well as optic pathway glioma, with a mutation of the MAPK/ERK pathway. Patient B is a 6 year old male with recurrent, refractory pilocytic astrocytoma of the optic pathway and hypothalamus, with progression through several courses of intravenous chemotherapy, noted to have somatic NACC2-NTRK mutation. Patient C is a 17 year old female with unresectable, pontomedullary ganglioglioma, noted to have BRAF-V600E mutation. Patient A is treated with Selumetinib, for about 6 months, with near resolution of nasopharyngeal mass. Patient B is treated with Larotrectinib, for about 3 months, with stability of clinical symptoms. Patient C is treated with Vemurafenib, for about 10 months, with stability of lesion size and patient-reported clinical improvement. No adverse events were noted for any of these patients and all medications were administered orally. Significant improvement in quality of life was reported, as they did not have central lines or bone marrow suppression. Targeted inhibitors provide a reasonable treatment option for relapsed, refractory CNS malignancies with actionable mutations.

Malignant transformation of an optic pathway glioma without prior radiation therapy

2010 ◽  
Vol 5 (5) ◽  
pp. 507-510 ◽  
Author(s):  
Garrett K. Zoeller ◽  
Carole D. Brathwaite ◽  
David I. Sandberg
Keyword(s):  
Radiation Therapy ◽  
English Literature ◽  
Optic Chiasm ◽  
External Beam Radiation ◽  
Optic Pathway Glioma ◽  
Malignant Degeneration ◽  
Low Grade ◽  
Optic Pathway ◽  
Beam Radiation ◽  
First Case

Optic pathway gliomas (OPGs) arise from the optic nerves, optic chiasm, and/or hypothalamus and most commonly occur in childhood. Although these tumors can be quite challenging to manage, they are typically low-grade astrocytomas histologically, most commonly pilocytic astrocytomas. The few previously reported cases of malignant degeneration of an OPG occurred after external beam radiation therapy. The authors report the first case in the English literature of an OPG that transformed from a low-grade astrocytoma, with features most consistent with a pilocytic astrocytoma, to a malignant glioma without any exposure to radiation therapy.

scholarly journals Disseminated craniospinal low-grade glioma in a patient with NF-1 without optic pathway pathology: illustrative case

2021 ◽  
Vol 2 (18) ◽  
Author(s):  
Alan R. Tang ◽  
Joseline Haizel-Cobbina ◽  
Paisit Paueksakon ◽  
Asha Sarma ◽  
Julie Bennett ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Molecular Testing ◽  
Illustrative Case ◽  
Low Grade ◽  
Optic Pathway ◽  
Autosomal Dominant Disorder ◽  
Imaging Evaluation ◽  
The Central Nervous System ◽  
The Right

BACKGROUND Neurofibromatosis type 1 (NF-1) is a neurocutaneous autosomal dominant disorder that predisposes patients to develop intracranial low-grade gliomas (LGGs). Most LGGs in patients with NF-1 involve the optic pathway but can arise anywhere throughout the central nervous system. NF-1–related disseminated pediatric LGG (dPLGG) in the absence of a dominant optic pathway glioma has not been described. OBSERVATIONS The authors discussed a case of a 10-year-old boy who presented with consideration for biopsy with nonoptic pathway PLGG with craniospinal dPLGG in the setting of NF-1. The patient’s primary lesion, located in the right medulla, was initially treated with surveillance before induction chemotherapy with carboplatin and vincristine was initiated. However, surveillance imaging demonstrated significant increase in size and enhancement, and subsequent craniospinal imaging demonstrated extensive nodular dissemination in the cervicothoracic spine. A biopsy and molecular testing were subsequently performed to further evaluate the tumor, and the patient was diagnosed with dPLGG with CDKN2A deletion. LESSONS Thorough craniospinal magnetic resonance imaging evaluation and biopsy in nonoptic pathway–dominant brain lesions in NF-1 are warranted in patients with atypical clinical and radiological findings in whom standard chemotherapeutic therapy fails.

scholarly journals C6 Cell Injection into the Optic Nerve of Long-Evans Rats: A Short-Term Model of Optic Pathway Gliomas

2020 ◽  
Vol 29 ◽  
pp. 096368972096438
Author(s):  
Deepti Singh ◽  
Pierre C. Dromel ◽  
Tatiana Perepelkina ◽  
Petr Baranov ◽  
Michael Young
Keyword(s):  
Optic Nerve ◽  
Glial Cells ◽  
Treatment Options ◽  
Genetic Defect ◽  
Tumor Formation ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway ◽  
Rat Glioma ◽  
Long Evans

The optic pathway glioma (OPG) is a slow-growing brain tumor that arises along the optic nerve or its downstream connections and causing vision to gradually worsen with time. This tumor forms in children with a genetic condition called neurofibromatosis type 1 (NF1), causing tumors to grow on nerves. In normal conditions, glial cells are there to support and protect nerve cells but, in NF1-OPG, glial cells have a genetic defect and grow out of control forming a tumor called a glioma. There are no rat models of NF1-OPG that can be used to explore various treatment options, and mouse models make interventional studies difficult due to their small eye size. We have created a model in which to study the progression of tumor growth in the optic nerve and establish the anatomical and functional consequences of the model and determine its suitability to serve as a surrogate for human disease. C6 rat glioma cells were injected into the optic nerve of Long-Evans rats and allowed to proliferate for 2 weeks. The eye clearly showed proptosis and lens opacity was observed, likely due to increased intraocular pressure caused by growing tumors. Hematoxylin–eosin staining showed marked cellularity, with hyperchromatism and pleomorphism. There was prominent area of necrosis with neoplastic cells palisading around the penumbra. Immunostaining with markers such as S100, β-tubulin III, Foxp3, CD45, Vimentin, and Ki67 confirmed low-grade tumor formation, with a mild immune response. Our results show the utility of a surgically induced rat model of OPG that may be used for exploring various treatment options for NF1 ocular tumors.

scholarly journals Predicting pediatric optic pathway glioma progression using advanced magnetic resonance image analysis and machine learning

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Jared M Pisapia ◽  
Hamed Akbari ◽  
Martin Rozycki ◽  
Jayesh P Thawani ◽  
Phillip B Storm ◽  
...  
Keyword(s):  
Machine Learning ◽  
Image Analysis ◽  
White Matter ◽  
Magnetic Resonance ◽  
Predictive Model ◽  
Diffusion Tensor ◽  
Machine Learning Techniques ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway

Abstract Background Optic pathway gliomas (OPGs) are low-grade tumors of the white matter of the visual system with a highly variable clinical course. The aim of the study was to generate a magnetic resonance imaging (MRI)-based predictive model of OPG tumor progression using advanced image analysis and machine learning techniques. Methods We performed a retrospective case–control study of OPG patients managed between 2009 and 2015 at an academic children’s hospital. Progression was defined as radiographic tumor growth or vision decline. To generate the model, optic nerves were manually highlighted and optic radiations (ORs) were segmented using diffusion tractography tools. For each patient, intensity distributions were obtained from within the segmented regions on all imaging sequences, including derivatives of diffusion tensor imaging (DTI). A machine learning algorithm determined the combination of features most predictive of progression. Results Nineteen OPG patients with progression were matched to 19 OPG patients without progression. The mean time between most recent follow-up and most recently analyzed MRI was 3.5 ± 1.7 years. Eighty-three MRI studies and 532 extracted features were included. The predictive model achieved an accuracy of 86%, sensitivity of 89%, and specificity of 81%. Fractional anisotropy of the ORs was among the most predictive features (area under the curve 0.83, P < 0.05). Conclusions Our findings show that image analysis and machine learning can be applied to OPGs to generate a MRI-based predictive model with high accuracy. As OPGs grow along the visual pathway, the most predictive features relate to white matter changes as detected by DTI, especially within ORs.

Visual outcomes after chemotherapy for optic pathway glioma in children with and without neurofibromatosis type 1: results of the International Society of Paediatric Oncology (SIOP) Low-Grade Glioma 2004 trial UK cohort

2018 ◽  
Vol 102 (10) ◽  
pp. 1367-1371 ◽  
Author(s):  
Kevin Falzon ◽  
Evangelos Drimtzias ◽  
Susan Picton ◽  
Ian Simmons
Keyword(s):  
Risk Factors ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Paediatric Oncology ◽  
Optic Pathway Glioma ◽  
Final Visit ◽  
Low Grade ◽  
Optic Pathway ◽  
Associated Risk Factors

AimsTo report visual acuity (VA) outcomes following chemotherapy for optic pathway glioma (OPG) in children with or without neurofibromatosis type-1 (NF1) and to analyse associated risk factors.MethodsA prospective, multicentre, cohort study involving 155 children treated between September 2004 and December 2012. Initial and final VA was used for per-eye and per-subject analysis. Correlation tests were performed to determine whether initial VA predicted final VA. Logistic regression was used to determine whether age and tumour location were associated risk factors.Results90 children had complete ophthalmological data. At initiation of chemotherapy, 26% and 49% of eyes with NF1-OPG and sporadic OPG, respectively, had VA of ≥0.7 log of the minimum angle of resolution (logMAR). At final visit, per eye, 49% had ≤0.2, 23% had 0.30–0.60 and 28% had VA≥0.70 logMAR in the NF1-OPG group. In the sporadic OPG group, per eye, 32% had ≤0.2, 11% had VA 0.30–0.60 and 57% had ≥0.70 logMAR. Children with sporadic OPG, per eye, were significantly less likely to have VA outcomes ≤0.60 logMAR compared with children with NF1-OPG (OR=0.30; 95% CI 0.16 to 0.56; P<0.0001). Per subject, VA improved in 24%, remained stable in 35% and worsened in 41% of children with NF1-OPG and improved in 18%, remained stable in 43% and worsened in 39% of children with sporadic OPG.ConclusionsChildren with and without NF1 demonstrated the same rate of VA improvement, stabilisation or worsening; however, children with sporadic OPG had a poorer VA outcome. Better initial VA, older age, absence of postchiasm tumour and presence of NF1 were associated with improved or stable VA outcomes.

Resolving mystery behind autonomous retrogression of low-grade gliomas; a systematic review

2020 ◽  
pp. 1-28
Author(s):  
Syed Ijlal Ahmed ◽  
Syeda Beenish Bareeqa ◽  
Syeda Sana Samar ◽  
Syed Daniyal Ahmed Jilanee
Keyword(s):  
Systematic Review ◽  
Therapeutic Intervention ◽  
Spontaneous Regression ◽  
Experimental Studies ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Evidence Based ◽  
Optic Pathway ◽  
Diffuse Astrocytoma ◽  
Low Grade Gliomas

Abstract Objective: To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome. Method: The systematic review comprised medical literature in English language published from January 1997 to January 2017 on Scopus, PubMed and Google Scholar databases to establish consensus about the possible mechanism of spontaneous regression, the role of therapeutic intervention and failure of management strategies in low-grade gliomas. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed during the review. Results: Of the 176 articles identified, 73(41.5%) were shortlisted for detailed assessment. Of them, 10(13.7%) were included; 5(50%) case reports and 5(50%) case series. There were 23 cases of spontaneous regression; 15(65.2%) males and 8(34.7%) females. The interval of regression varied from 3 months to 15.5 years, and the most commonly presenting low-grade glioma type was optic pathway glioma 11(47.4%). Conclusion: The phenomenon of regression was most evident in optic pathway glioma. Literature suggested that low-grade gliomas should undergo serial imaging before implying any therapeutic intervention. However, the evidence-based proof, large-scale experimental studies and ethical considerations are still required to standardise this strategy. Key Words: Pilocytic astrocytomas, Desmoplastic infantile ganglioglioma, DIG, Desmoplastic infantile astrocytomas, DIA, Diffuse astrocytoma, Spontaneous regression. Continuous...

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