Optic Pathway Glioma disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: OPT032 MIFTS: 34	Optic Pathway Glioma Categories: Endocrine diseases, Eye diseases, Neuronal diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
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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:

MalaCards categories:
Global: Rare diseases
Anatomical: Neuronal diseases Endocrine diseases Eye diseases

See all MalaCards categories (disease lists)

ICD10: ³²

Neoplasms

Benign neoplasms

Benign neoplasm of brain and other parts of central nervous system

Benign neoplasm: Cranial nerves

Orphanet: ⁵⁸

Rare neurological diseases

Rare endocrine diseases

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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).

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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
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:

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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%)

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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

Vincristine

Approved, Investigational

Phase 3

2068-78-2, 57-22-7

5978

Antimitotic Agents

Phase 3

Tubulin Modulators

Phase 3

Irinotecan

Approved, Investigational

Phase 2

97682-44-5, 100286-90-6

60838

Lenalidomide

Approved

Phase 2

191732-72-6

216326

topoisomerase I inhibitors

Phase 2

Immunologic Factors

Phase 2

Angiogenesis Inhibitors

Phase 2

Vorinostat

Approved, Investigational

Phase 1

149647-78-9

5311

Bortezomib

Approved, Investigational

Phase 1

179324-69-7

387447 93860

Oxaliplatin

Approved, Investigational

Phase 1

61825-94-3

43805 11947679 6857599

Pomalidomide

Approved

Phase 1

19171-19-8

134780

Busulfan

Approved, Investigational

Phase 1

55-98-1

2478

Melphalan

Approved

Phase 1

148-82-3

4053 460612

Lenograstim

Approved, Investigational

Phase 1

135968-09-1

Topotecan

Approved, Investigational

Phase 1

123948-87-8, 119413-54-6

60699 60700

Mechlorethamine

Approved, Investigational

Phase 1

51-75-2

4033

Dexamethasone acetate

Approved, Investigational, Vet_approved

Phase 1

1177-87-3

3680

Dexamethasone

Approved, Investigational, Vet_approved

Phase 1

50-02-2

3003 5743

D-Phenylalanine

Approved, Experimental, Investigational, Nutraceutical

Phase 1

63-91-2, 673-06-3

6140 71567

Entinostat

Investigational

Phase 1

209783-80-2

4261

Alvocidib

Experimental, Investigational

Phase 1

131740-09-5, 146426-40-6

5466794 5287969

Cediranib

Investigational

Phase 1

288383-20-0

9933475

Maleic acid

Experimental, Investigational

Phase 1

110-16-7, 110-17-8

444266 444972

Histone Deacetylase Inhibitors

Phase 1

Protein Kinase Inhibitors

Phase 1

Adjuvants, Immunologic

Phase 1

Immunosuppressive Agents

Phase 1

Antineoplastic Agents, Hormonal

Phase 1

Gastrointestinal Agents

Phase 1

R04929097

Phase 1

Hormones

Phase 1

Hormone Antagonists

Phase 1

Antiemetics

Phase 1

glucocorticoids

Phase 1

BB 1101

Phase 1

Anti-Inflammatory Agents

Phase 1

Dopamine

Approved

62-31-7, 51-61-6

681

Dihydroxyphenylalanine

Neurotransmitter Agents

Dopamine Agents

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

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Genetic Tests for Optic Pathway Glioma

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Anatomical Context for Optic Pathway Glioma

Organs/tissues related to Optic Pathway Glioma:

MalaCards : Brain, Small Intestine, Pituitary, Colon

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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	Cantor E...Brossier NM	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	Rask O...Swedish Childhood CNS Tumor Working Group (VCTB)	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	Pinheiro SL...Simoes-Pereira J	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	Green K...Opocher E	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	Rony C...Halevy A	35381604	2022
6	Management of Optic Pathway Glioma: A Systematic Review and Meta-Analysis. ⁶²	Yousefi O...Borghei-Razavi H	36230704	2022
7	Secondary Narcolepsy as Worsening Sign in a Pediatric Case of Optic Pathway Glioma. ⁶²	Laus B...Cacchione A	36291391	2022
8	Trans-eyebrow supraorbital endoscope-assisted keyhole approach to suprasellar meningioma in pediatric patient: case report and literature review. ⁶²	Safronova EI...Kushel YV	36104809	2022
9	Risk factors for treatment-refractory and relapsed optic pathway glioma in children with neurofibromatosis type 1. ⁶²	Kotch C...Fisher MJ	35018469	2022
10	Glioma of the optic nerve and chiasm: a case report. ⁶²	Pas CB...Brito AG	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	Liu ZM...Tian YJ	35106677	2022
12	Optic pathway glioma and endocrine disorders in patients with and without NF1. ⁶²	Gil Margolis M...Shalitin S	35538247	2022
13	Imaging Findings of Pediatric Orbital Masses and Tumor Mimics. ⁶²	Joseph AK...Silvera VM	35245105	2022
14	Identification of three clinical neurofibromatosis 1 subtypes: Latent class analysis of a series of 1351 patients. ⁶²	Bergqvist C...NF France	35098592	2022
15	Visual outcomes and predictors in optic pathway glioma: a single centre study. ⁶²	Bowman R...Thompson DA	35562551	2022
16	Recent Updates on Radiation Therapy for Pediatric Optic Pathway Glioma. ⁶²	Kim N...Lim DH	35545828	2022
17	Neurofibromatosis Type 1 Has a Wide Spectrum of Growth Hormone Excess. ⁶²	Hannah-Shmouni F...Stratakis CA	35456261	2022
18	Recent Update in Pharmacological Agents for Optic Pathway Glioma. ⁶²	Park M	35545829	2022
19	Visual Outcomes of Optic Pathway Glioma Treated With Chemotherapy in Neurofibromatosis Type 1. ⁶²	Jose P...Jorge Teixeira F	34592874	2022
20	Regression and pseudoprogression of pediatric optic pathway glioma in patients treated with proton beam therapy. ⁶²	Youn SH...Kim JY	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	Kilian A...Tietze A	34994964	2022
22	[Prognostic factors of neurofibromatosis type 1-associated optic pathway gliomas in children]. ⁶²	Florent C...Denis D	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	Kang E...Lee BH	35093157	2022
24	Novel "T-Dimension" Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend. ⁶²	Giotta Lucifero A...Luzzi S	35588700	2022
25	A Single-Center Treatment Experience of Gamma Knife Radiosurgery for Optic Pathway Glioma. ⁶²	Ge Y...Liu D	35983244	2022
26	Editorial: Optic pathway glioma: A multidisciplinary entity, posing dilemmas in diagnosis and management. ⁶²	Habibi Z...Zanon N	35990103	2022
27	Optical Coherence Tomography Identifies Visual Pathway Involvement Earlier than Visual Function Tests in Children with MRI-Verified Optic Pathway Gliomas. ⁶²	Arnljots U...Hellgren K	35053482	2022
28	Neurosurgery for Optic Pathway Glioma: Optimizing Multidisciplinary Management. ⁶²	Samples DC...Hankinson TC	35599811	2022
29	Current update on the visual outcome of optic pathway glioma associated with neurofibromatosis type-1. ⁶²	Zeid JL	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	Lohkamp LN...Rutka JT	35592129	2022
31	Mutational spectrum of NF1 gene in 24 unrelated Egyptian families with neurofibromatosis type 1. ⁶²	N Abdel-Aziz N...Amr KS	34080803	2021
32	Asthma reduces glioma formation by T cell decorin-mediated inhibition of microglia. ⁶²	Chatterjee J...Gutmann DH	34880260	2021
33	Predictive Modeling for Clinical Features Associated With Neurofibromatosis Type 1. ⁶²	Morris SM...Payne PRO	34987881	2021
34	Disseminated craniospinal low-grade glioma in a patient with NF-1 without optic pathway pathology: illustrative case. ⁶²	Tang AR...Dewan MC	36061627	2021
35	Optic pathway glioma and the sex association in neurofibromatosis type 1: a single-center study. ⁶²	Henning AM...Ejerskov C	34809690	2021
36	Inhibition of ERK/MAPK signaling as potential therapy to prevent optic pathway glioma in infants with neurofibromatosis type 1. ⁶²	D'Angelo F...Lasorella A	34699786	2021
37	Selumetinib for optic pathway glioma: Seeing through the fog, (not yet) the end of the tunnel? ⁶²	Ramaswamy V...Bartels U	34251024	2021
38	A Unique Case of Sporadic Optic Pathway Glioma in an Infant With Acute Nystagmus. ⁶²	Knight CT...Standley T	34646624	2021
39	Neurofibromatosis Type 1 in Children: A Single-Center Experience. ⁶²	Gonca Kacar A...Tiraje Celkan T	35005728	2021
40	Postoperative hydrocephalus is a high-risk lethal factor for patients with low-grade optic pathway glioma. ⁶²	Han S...Yu C	34240664	2021
41	Early radiotherapy preserves vision in sporadic optic pathway glioma. ⁶²	Hanania AN...Chintagumpala MM	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	Vagge A...Traverso CE	32066268	2021
43	A therapeutic window for preventive therapy in NF1-associated optic pathway glioma. ⁶²	Zhu Y...Treisman DM	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	Nuijts MA...Stegeman I	34941930	2021
45	Neuroimaging Features of Optic Nerve Hemangioblastoma Identified by Conventional and Advanced Magnetic Resonance Techniques: A Case Report and Literature Review. ⁶²	Duan M...Feng M	34868983	2021
46	NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat. ⁶²	Azizi AA...SIOPE NF1 OPG Nottingham, UK, Workshop 2014	32628746	2021
47	Impact of systemic anticancer therapy in pediatric optic pathway glioma on visual function: A systematic review. ⁶²	Bennebroek CAM...Saeed P	34673789	2021
48	Neurofibromatosis Type 1-Associated Optic Pathway Glioma in Children: A Follow-Up of 10 Years or More. ⁶²	Kinori M...Zeid JL	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	Pajavand AM...Meybodi TE	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	Kandels D...Gnekow AK	32580249	2020