MCID: OPT032
MIFTS: 34

Optic Pathway Glioma

Categories: Endocrine diseases, Eye diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Optic Pathway Glioma

MalaCards integrated aliases for Optic Pathway Glioma:

Name: Optic Pathway Glioma 19 58
Visual Pathway Glioma 71

Characteristics:


Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare endocrine diseases


Summaries for Optic Pathway Glioma

GARD: 19 Optic pathway glioma (OPG) is a benign tumor that develop along the optic nerve (chiasm, tracts, and radiations) characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. OPG are often linked to neurofibromatosis type 1 (NF1, see this term).

MalaCards based summary: Optic Pathway Glioma, also known as visual pathway glioma, is related to glioma susceptibility 1 and glioma. The drugs Vincristine and Antimitotic Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, small intestine and pituitary, and related phenotypes are nystagmus and optic atrophy

Orphanet: 58 Optic pathway glioma (OPG) is a benign tumor that develop along the optic nerve (chiasm, tracts, and radiations) characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. OPG are often linked to neurofibromatosis type 1 (NF1, see this term).

Related Diseases for Optic Pathway Glioma

Diseases related to Optic Pathway Glioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 81)
# Related Disease Score Top Affiliating Genes
1 glioma susceptibility 1 11.0
2 glioma 11.0
3 glial tumor 11.0
4 neurofibromatosis 10.9
5 neurofibromatosis, type i 10.9
6 neurofibromatosis-noonan syndrome 10.9
7 pilocytic astrocytoma 10.4
8 strabismus 10.3
9 low grade glioma 10.3
10 bap1 tumor predisposition syndrome 10.3
11 inherited cancer-predisposing syndrome 10.3
12 factor vii deficiency 10.3
13 brain cancer 10.3
14 hydrocephalus 10.2
15 high grade glioma 10.2
16 optic nerve glioma 10.2
17 precocious puberty 10.2
18 rare tumor 10.2
19 3-methylglutaconic aciduria, type iii 10.1
20 congenital hemidysplasia with ichthyosiform erythroderma and limb defects 10.1
21 scoliosis 10.1
22 central precocious puberty 10.1
23 amblyopia 10.1
24 exotropia 10.1
25 acromegaly 10.1
26 syringomyelia 10.1
27 central nervous system cancer 10.1
28 juvenile pilocytic astrocytoma 10.1
29 intracranial hypertension 10.1
30 pathologic nystagmus 10.1
31 children's interstitial lung disease 10.1
32 diencephalic syndrome 10.1
33 low-grade astrocytoma 10.1
34 idiopathic syringomyelia 10.1
35 coloboma of optic nerve 10.0
36 hypogonadotropic hypogonadism 7 with or without anosmia 10.0
37 nevus, epidermal 10.0
38 obsessive-compulsive disorder 10.0
39 retinoblastoma 10.0
40 hypothyroidism, congenital, nongoitrous, 2 10.0
41 hypoascorbemia 10.0
42 moyamoya disease 1 10.0
43 retinitis pigmentosa 10.0
44 rhabdomyosarcoma 2 10.0
45 mismatch repair cancer syndrome 1 10.0
46 meningioma, familial 10.0
47 hypothyroidism, congenital, nongoitrous, 3 10.0
48 intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies 10.0
49 deficiency anemia 10.0
50 hypogonadotropic hypogonadism 10.0

Graphical network of the top 20 diseases related to Optic Pathway Glioma:



Diseases related to Optic Pathway Glioma

Symptoms & Phenotypes for Optic Pathway Glioma

Human phenotypes related to Optic Pathway Glioma:

58 30 (show all 23)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nystagmus 58 30 Frequent (33%) Frequent (79-30%)
HP:0000639
2 optic atrophy 58 30 Frequent (33%) Frequent (79-30%)
HP:0000648
3 neurofibromas 58 30 Frequent (33%) Frequent (79-30%)
HP:0001067
4 visual loss 58 30 Frequent (33%) Frequent (79-30%)
HP:0000572
5 seizure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001250
6 hydrocephalus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000238
7 precocious puberty 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000826
8 developmental regression 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002376
9 global developmental delay 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001263
10 blindness 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000618
11 vomiting 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002013
12 strabismus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000486
13 growth delay 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001510
14 ophthalmoplegia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000602
15 vertigo 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002321
16 proptosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000520
17 headache 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002315
18 visual field defect 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001123
19 fatigable weakness 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003473
20 nausea 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002018
21 papilledema 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001085
22 reduced visual acuity 58 Frequent (79-30%)
23 abnormality of the nervous system 58 Occasional (29-5%)

Drugs & Therapeutics for Optic Pathway Glioma

Drugs for Optic Pathway Glioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
2 Antimitotic Agents Phase 3
3 Tubulin Modulators Phase 3
4
Irinotecan Approved, Investigational Phase 2 97682-44-5, 100286-90-6 60838
5
Lenalidomide Approved Phase 2 191732-72-6 216326
6 topoisomerase I inhibitors Phase 2
7 Immunologic Factors Phase 2
8 Angiogenesis Inhibitors Phase 2
9
Vorinostat Approved, Investigational Phase 1 149647-78-9 5311
10
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
11
Oxaliplatin Approved, Investigational Phase 1 61825-94-3 43805 11947679 6857599
12
Pomalidomide Approved Phase 1 19171-19-8 134780
13
Busulfan Approved, Investigational Phase 1 55-98-1 2478
14
Melphalan Approved Phase 1 148-82-3 4053 460612
15
Lenograstim Approved, Investigational Phase 1 135968-09-1
16
Topotecan Approved, Investigational Phase 1 123948-87-8, 119413-54-6 60699 60700
17
Mechlorethamine Approved, Investigational Phase 1 51-75-2 4033
18
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 1 1177-87-3 3680
19
Dexamethasone Approved, Investigational, Vet_approved Phase 1 50-02-2 3003 5743
20
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 1 63-91-2, 673-06-3 6140 71567
21
Entinostat Investigational Phase 1 209783-80-2 4261
22
Alvocidib Experimental, Investigational Phase 1 131740-09-5, 146426-40-6 5466794 5287969
23
Cediranib Investigational Phase 1 288383-20-0 9933475
24
Maleic acid Experimental, Investigational Phase 1 110-16-7, 110-17-8 444266 444972
25 Histone Deacetylase Inhibitors Phase 1
26 Protein Kinase Inhibitors Phase 1
27 Adjuvants, Immunologic Phase 1
28 Immunosuppressive Agents Phase 1
29 Antineoplastic Agents, Hormonal Phase 1
30 Gastrointestinal Agents Phase 1
31 R04929097 Phase 1
32 Hormones Phase 1
33 Hormone Antagonists Phase 1
34 Antiemetics Phase 1
35 glucocorticoids Phase 1
36 BB 1101 Phase 1
37 Anti-Inflammatory Agents Phase 1
38
Dopamine Approved 62-31-7, 51-61-6 681
39 Dihydroxyphenylalanine
40 Neurotransmitter Agents
41 Dopamine Agents
42 Fluorides

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 A Phase 3 Randomized Study of Selumetinib Versus Carboplatin/Vincristine in Newly Diagnosed or Previously Untreated Neurofibromatosis Type 1 (NF1) Associated Low-Grade Glioma (LGG) Recruiting NCT03871257 Phase 3 Carboplatin;Selumetinib Sulfate;Vincristine Sulfate
2 A Randomized Phase 3 Study of Antineoplastons A10 and AS2-1 vs. Temozolomide in Subjects With Recurrent and / or Progressive Optic Pathway Glioma After Carboplatin or Cisplatin Therapy Withdrawn NCT01260103 Phase 3 Temozolomide;ANP Therapy
3 Phase II Study of Antineoplastons A10 and AS2-1 Infusions in Children With Visual Pathway Glioma Completed NCT00003477 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
4 Phase II Trial of Irinotecan in Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
5 A Paediatric Phase I/II Study Of Intermittent Dosing Of The Mek-1 Inhibitor Selumetinib In Children With Neurofibromatosis Type-1 And Inoperable Plexiform Neurofibroma And/Or Progressive Optic Pathway Glioma Recruiting NCT03326388 Phase 1, Phase 2 Selumetinib
6 A Phase 1 and Phase II and Re-Treatment Study of AZD6244 for Recurrent or Refractory Pediatric Low Grade Glioma Active, not recruiting NCT01089101 Phase 1, Phase 2 Selumetinib
7 A Phase II Randomized Trial of Lenalidomide (NSC # 703813) in Pediatric Patients With Recurrent, Refractory or Progressive Juvenile Pilocytic Astrocytomas and Optic Pathway Gliomas Active, not recruiting NCT01553149 Phase 2 Lenalidomide
8 A Phase 1 Study of Entinostat, an Oral Histone Deacetylase Inhibitor, in Pediatric Patients With Recurrent or Refractory Solid Tumors, Including CNS Tumors and Lymphoma Completed NCT02780804 Phase 1 Entinostat
9 A PHASE I STUDY OF FLAVOPIRIDOL (NSC# 649890; IND# 46211) IN PATIENTS WITH RELAPSED OR REFRACTORY PEDIATRIC SOLID TUMORS OR LYMPHOMAS Completed NCT00012181 Phase 1 alvocidib
10 A Phase I Study of Vorinostat and Bortezomib in Children With Refractory or Recurrent Solid Tumors, Including CNS Tumors and Lymphomas Completed NCT00994500 Phase 1 vorinostat;bortezomib
11 A Phase I Study of Oxaliplatin (NSC# 266046, IND #57004) and Irinotecan in Pediatric Patients With Refractory Solid Tumors and Lymphomas Completed NCT00101270 Phase 1 irinotecan hydrochloride;oxaliplatin
12 A Phase I Study of Pazopanib as a Single Agent for Children With Relapsed or Refractory Solid Tumors Completed NCT00929903 Phase 1 pazopanib hydrochloride
13 A Phase I Clinical Trial of AZD2171 in Children With Recurrent or Progressive Central Nervous System (CNS) Tumors Completed NCT00326664 Phase 1 Cediranib Maleate
14 A Phase I Trial of Pomalidomide for Children With Recurrent, Progressive, or Refractory CNS Tumors Completed NCT02415153 Phase 1 Pomalidomide
15 Pilot Study of High-Dose Chemotherapy With Busulfan, Melphalan, and Topotecan Followed by Autologous Hematopoietic Stem Cell Transplant in Advanced Stage and Recurrent Tumors Active, not recruiting NCT00638898 Phase 1 busulfan;melphalan;topotecan hydrochloride
16 A PHASE 1/2 STUDY OF RO4929097, AN ORAL SMALL MOLECULE INHIBITOR OF GAMMA-SECRETASE, IN CHILDREN WITH RELAPSED/REFRACTORY SOLID OR CNS TUMORS, LYMPHOMA, OR T-CELL LEUKEMIA Terminated NCT01088763 Phase 1 gamma-secretase/Notch signalling pathway inhibitor RO4929097;dexamethasone
17 A Children's Oncology Group Protocol for Collecting and Banking Pediatric Brain Tumor Research Specimens Completed NCT00919750
18 A Phase II Study of Hippocampal-Avoidance Using Proton Therapy in Low-Grade Glioma Recruiting NCT04065776
19 Hypotonia as a Clinical Predictor of Optic Pathway Glioma in Children With Neurofibromatosis Type 1 Terminated NCT02584413 Gadolinium contrast
20 18F-FDOPA PET/CT or PET/MRI in Patients With Gliomas Terminated NCT02175745 18F-fluoro-dihydroxyphenylalanine
21 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452

Search NIH Clinical Center for Optic Pathway Glioma

Genetic Tests for Optic Pathway Glioma

Anatomical Context for Optic Pathway Glioma

Organs/tissues related to Optic Pathway Glioma:

MalaCards : Brain, Small Intestine, Pituitary, Colon

Publications for Optic Pathway Glioma

Articles related to Optic Pathway Glioma:

(show top 50) (show all 269)
# Title Authors PMID Year
1
Dose-dependent seizure control with MEK inhibitor therapy for progressive glioma in a child with neurofibromatosis type 1. 62
35648241 2022
2
Prospective registration of symptoms and times to diagnosis in children and adolescents with central nervous system tumors: A study of the Swedish Childhood Cancer Registry. 62
35727740 2022
3
Precocious and accelerated puberty in children with neurofibromatosis type 1: results from a close follow-up of a cohort of 45 patients. 62
36269545 2022
4
A Nationwide Evaluation of Bevacizumab-based Treatments in Paediatric Low-Grade Glioma in the UK: Safety. Efficacy, Visual Morbidity and Outcomes. 62
36239316 2022
5
The Utility of Early Brain MRI for Patients with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Long-Term Follow-Up in a Tertiary Referral Hospital. 62
35381604 2022
6
Management of Optic Pathway Glioma: A Systematic Review and Meta-Analysis. 62
36230704 2022
7
Secondary Narcolepsy as Worsening Sign in a Pediatric Case of Optic Pathway Glioma. 62
36291391 2022
8
Trans-eyebrow supraorbital endoscope-assisted keyhole approach to suprasellar meningioma in pediatric patient: case report and literature review. 62
36104809 2022
9
Risk factors for treatment-refractory and relapsed optic pathway glioma in children with neurofibromatosis type 1. 62
35018469 2022
10
Glioma of the optic nerve and chiasm: a case report. 62
35857979 2022
11
The role of imaging features and resection status in the survival outcome of sporadic optic pathway glioma children receiving different adjuvant treatments. 62
35106677 2022
12
Optic pathway glioma and endocrine disorders in patients with and without NF1. 62
35538247 2022
13
Imaging Findings of Pediatric Orbital Masses and Tumor Mimics. 62
35245105 2022
14
Identification of three clinical neurofibromatosis 1 subtypes: Latent class analysis of a series of 1351 patients. 62
35098592 2022
15
Visual outcomes and predictors in optic pathway glioma: a single centre study. 62
35562551 2022
16
Recent Updates on Radiation Therapy for Pediatric Optic Pathway Glioma. 62
35545828 2022
17
Neurofibromatosis Type 1 Has a Wide Spectrum of Growth Hormone Excess. 62
35456261 2022
18
Recent Update in Pharmacological Agents for Optic Pathway Glioma. 62
35545829 2022
19
Visual Outcomes of Optic Pathway Glioma Treated With Chemotherapy in Neurofibromatosis Type 1. 62
34592874 2022
20
Regression and pseudoprogression of pediatric optic pathway glioma in patients treated with proton beam therapy. 62
34766717 2022
21
Tumor load rather than contrast enhancement is associated with the visual function of children and adolescents with optic pathway glioma - a retrospective Magnetic Resonance Imaging study. 62
34994964 2022
22
[Prognostic factors of neurofibromatosis type 1-associated optic pathway gliomas in children]. 62
34972579 2022
23
Whole-body MRI evaluation in neurofibromatosis type 1 patients younger than 3 years old and the genetic contribution to disease progression. 62
35093157 2022
24
Novel "T-Dimension" Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend. 62
35588700 2022
25
A Single-Center Treatment Experience of Gamma Knife Radiosurgery for Optic Pathway Glioma. 62
35983244 2022
26
Editorial: Optic pathway glioma: A multidisciplinary entity, posing dilemmas in diagnosis and management. 62
35990103 2022
27
Optical Coherence Tomography Identifies Visual Pathway Involvement Earlier than Visual Function Tests in Children with MRI-Verified Optic Pathway Gliomas. 62
35053482 2022
28
Neurosurgery for Optic Pathway Glioma: Optimizing Multidisciplinary Management. 62
35599811 2022
29
Current update on the visual outcome of optic pathway glioma associated with neurofibromatosis type-1. 62
36117804 2022
30
Optic Pathway Glioma in Children with Neurofibromatosis Type 1: A Multidisciplinary Entity, Posing Dilemmas in Diagnosis and Management Multidisciplinary Management of Optic Pathway Glioma in Children with Neurofibromatosis Type 1. 62
35592129 2022
31
Mutational spectrum of NF1 gene in 24 unrelated Egyptian families with neurofibromatosis type 1. 62
34080803 2021
32
Asthma reduces glioma formation by T cell decorin-mediated inhibition of microglia. 62
34880260 2021
33
Predictive Modeling for Clinical Features Associated With Neurofibromatosis Type 1. 62
34987881 2021
34
Disseminated craniospinal low-grade glioma in a patient with NF-1 without optic pathway pathology: illustrative case. 62
36061627 2021
35
Optic pathway glioma and the sex association in neurofibromatosis type 1: a single-center study. 62
34809690 2021
36
Inhibition of ERK/MAPK signaling as potential therapy to prevent optic pathway glioma in infants with neurofibromatosis type 1. 62
34699786 2021
37
Selumetinib for optic pathway glioma: Seeing through the fog, (not yet) the end of the tunnel? 62
34251024 2021
38
A Unique Case of Sporadic Optic Pathway Glioma in an Infant With Acute Nystagmus. 62
34646624 2021
39
Neurofibromatosis Type 1 in Children: A Single-Center Experience. 62
35005728 2021
40
Postoperative hydrocephalus is a high-risk lethal factor for patients with low-grade optic pathway glioma. 62
34240664 2021
41
Early radiotherapy preserves vision in sporadic optic pathway glioma. 62
33739455 2021
42
Role of visual evoked potentials and optical coherence tomography in the screening for optic pathway gliomas in patients with neurofibromatosis type I. 62
32066268 2021
43
A therapeutic window for preventive therapy in NF1-associated optic pathway glioma. 62
35419473 2021
44
The diagnostic accuracy and prognostic value of OCT for the evaluation of the visual function in children with a brain tumour: A systematic review. 62
34941930 2021
45
Neuroimaging Features of Optic Nerve Hemangioblastoma Identified by Conventional and Advanced Magnetic Resonance Techniques: A Case Report and Literature Review. 62
34868983 2021
46
NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat. 62
32628746 2021
47
Impact of systemic anticancer therapy in pediatric optic pathway glioma on visual function: A systematic review. 62
34673789 2021
48
Neurofibromatosis Type 1-Associated Optic Pathway Glioma in Children: A Follow-Up of 10 Years or More. 62
32283094 2021
49
Case Report: Chemotherapy Indication in a Case of Neurofibromatosis Type 1 Presenting Optic Pathway Glioma: A One-Year Clinical Case Study Using Differential Tractography Approach. 62
33994974 2021
50
Loss of efficacy of subsequent nonsurgical therapy after primary treatment failure in pediatric low-grade glioma patients-Report from the German SIOP-LGG 2004 cohort. 62
32580249 2020

Variations for Optic Pathway Glioma

Expression for Optic Pathway Glioma

Search GEO for disease gene expression data for Optic Pathway Glioma.

Pathways for Optic Pathway Glioma

GO Terms for Optic Pathway Glioma

Sources for Optic Pathway Glioma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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