MCID: PLM021
MIFTS: 37

Pilomyxoid Astrocytoma

Categories: Neuronal diseases, Rare diseases, Cancer diseases

Aliases & Classifications for Pilomyxoid Astrocytoma

MalaCards integrated aliases for Pilomyxoid Astrocytoma:

Name: Pilomyxoid Astrocytoma 12 59 15 73

Characteristics:

Orphanet epidemiological data:

59
pilomyxoid astrocytoma
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4845
NCIt 50 C40315
SNOMED-CT 68 388600004
Orphanet 59 ORPHA251615
UMLS via Orphanet 74 C1519086
ICD10 via Orphanet 34 C71.9
UMLS 73 C1519086

Summaries for Pilomyxoid Astrocytoma

MalaCards based summary : Pilomyxoid Astrocytoma is related to pilocytic astrocytoma and angiocentric glioma. An important gene associated with Pilomyxoid Astrocytoma is SYP (Synaptophysin), and among its related pathways/superpathways are Neural Stem Cell Differentiation Pathways and Lineage-specific Markers and Cell adhesion_ECM remodeling. The drugs Cyproheptadine and Histamine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and cerebellum, and related phenotypes are adipose tissue and digestive/alimentary

Related Diseases for Pilomyxoid Astrocytoma

Diseases related to Pilomyxoid Astrocytoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 80)
# Related Disease Score Top Affiliating Genes
1 pilocytic astrocytoma 31.4 GFAP KIAA1549 SYP
2 angiocentric glioma 30.3 GFAP SYP
3 ependymoma 29.9 GFAP SYP
4 astrocytoma 10.9
5 tympanosclerosis 10.4 COL1A1 COL2A1
6 cerebellum cancer 10.4 SUFU SYP
7 synovial chondromatosis 10.4 COL1A1 COL2A1
8 skin sarcoma 10.4 COL1A1 SYP
9 atypical choroid plexus papilloma 10.3 SUFU SYP
10 desmoplastic infantile ganglioglioma 10.3 GFAP SYP
11 extraventricular neurocytoma 10.3 GFAP SYP
12 lung combined type small cell carcinoma 10.3 GFAP SYP
13 cellular ependymoma 10.3 GFAP SYP
14 gangliocytoma 10.3 GFAP SYP
15 rhabdoid meningioma 10.3 GFAP SYP
16 chordoid meningioma 10.3 GFAP SYP
17 central nervous system primitive neuroectodermal neoplasm 10.3 GFAP SYP
18 subependymal glioma 10.3 GFAP SYP
19 chiari malformation 10.3 GFAP SYP
20 dysembryoplastic neuroepithelial tumor 10.3 GFAP SYP
21 cerebral neuroblastoma 10.3 GFAP SYP
22 benign ependymoma 10.3 GFAP SYP
23 astroblastoma 10.3 GFAP SYP
24 cerebral ventricle cancer 10.3 GFAP SYP
25 cerebrum cancer 10.3 GFAP SYP
26 pineal gland cancer 10.3 GFAP SYP
27 melanotic neuroectodermal tumor 10.3 GFAP SYP
28 obstructive hydrocephalus 10.3 GFAP SYP
29 supratentorial cancer 10.3 GFAP SYP
30 medulloepithelioma 10.3 GFAP SYP
31 pineocytoma 10.3 GFAP SYP
32 subependymoma 10.2 GFAP SYP
33 anaplastic ependymoma 10.2 GFAP SYP
34 tanycytic ependymoma 10.2 GFAP SYP
35 papillary ependymoma 10.2 GFAP SYP
36 spondyloepiphyseal dysplasia congenita 10.2 COL1A1 COL2A1
37 pleomorphic xanthoastrocytoma 10.2 GFAP SYP
38 pineoblastoma 10.2 GFAP SYP
39 subependymal giant cell astrocytoma 10.2 GFAP SYP
40 bone development disease 10.2 COL1A1 COL2A1
41 papilloma of choroid plexus 10.2 GFAP SYP
42 diencephalic syndrome 10.2
43 central neurocytoma 10.2 GFAP SYP
44 binswanger's disease 10.2 GFAP SYP
45 collagen disease 10.2 COL1A1 COL2A1
46 cystic teratoma 10.2 GFAP SYP
47 cerebellopontine angle tumor 10.2 GFAP SYP
48 medullomyoblastoma 10.1 GFAP SYP
49 clear cell ependymoma 10.1 GFAP SYP
50 ganglioneuroma 10.1 GFAP SYP

Graphical network of the top 20 diseases related to Pilomyxoid Astrocytoma:



Diseases related to Pilomyxoid Astrocytoma

Symptoms & Phenotypes for Pilomyxoid Astrocytoma

MGI Mouse Phenotypes related to Pilomyxoid Astrocytoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 adipose tissue MP:0005375 9.46 COL1A1 COL2A1 KIAA1549 SUFU
2 digestive/alimentary MP:0005381 9.26 COL1A1 COL2A1 GFAP SUFU
3 normal MP:0002873 9.02 COL1A1 COL2A1 GFAP SUFU SYP

Drugs & Therapeutics for Pilomyxoid Astrocytoma

Drugs for Pilomyxoid Astrocytoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 47)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyproheptadine Approved Phase 3 129-03-3 2913
2
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
3 Dermatologic Agents Phase 3,Phase 1
4
Histamine Phosphate Phase 3 51-74-1 65513
5 Serotonin Agents Phase 3
6 Serotonin Antagonists Phase 3
7 Gastrointestinal Agents Phase 3,Not Applicable
8 Neurotransmitter Agents Phase 3,Not Applicable
9 Histamine Antagonists Phase 3
10 Histamine H1 Antagonists Phase 3
11 Anti-Allergic Agents Phase 3
12 Antipruritics Phase 3
13
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
14
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
15
Dacarbazine Approved, Investigational Phase 1, Phase 2 4342-03-4 5351166
16
Irinotecan Approved, Investigational Phase 1, Phase 2 97682-44-5, 100286-90-6 60838
17
Vincristine Approved, Investigational Phase 1, Phase 2 2068-78-2, 57-22-7 5978
18
Temozolomide Approved, Investigational Phase 1, Phase 2 85622-93-1 5394
19
Cetuximab Approved Phase 1, Phase 2 205923-56-4 56842117 2333
20
Piperazine Approved, Vet_approved Phase 1, Phase 2 110-85-0 4837
21
Mebendazole Approved, Vet_approved Phase 1, Phase 2 31431-39-7 4030
22
Bevacizumab Approved, Investigational Phase 1, Phase 2,Phase 2 216974-75-3
23
Crizotinib Approved Phase 1, Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
24
Citric Acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 77-92-9 311
25 Alkylating Agents Phase 1, Phase 2
26 topoisomerase I inhibitors Phase 1, Phase 2
27 Topoisomerase Inhibitors Phase 1, Phase 2
28 Angiogenesis Inhibitors Phase 1, Phase 2,Phase 2
29 Angiogenesis Modulating Agents Phase 1, Phase 2,Phase 2
30 Anthelmintics Phase 1, Phase 2
31 Piperazine citrate Phase 1, Phase 2
32 Anticonvulsants Phase 1, Phase 2
33 Anti-Infective Agents Phase 1, Phase 2
34 Antiparasitic Agents Phase 1, Phase 2
35 Antimitotic Agents Phase 1, Phase 2
36 Antineoplastic Agents, Alkylating Phase 1, Phase 2
37 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
38 Protein Kinase Inhibitors Phase 1, Phase 2
39 Citrate Nutraceutical Phase 1, Phase 2
40
Verteporfin Approved, Investigational Phase 1 129497-78-5
41
Trioxsalen Approved Phase 1 3902-71-4 5585
42 Dihematoporphyrin Ether Phase 1
43 Photosensitizing Agents Phase 1
44
Dopamine Approved Not Applicable 51-61-6, 62-31-7 681
45 Fluorides Not Applicable
46 Dihydroxyphenylalanine Not Applicable
47 Dopamine Agents Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
2 A Phase I Study of Mebendazole for the Treatment of Pediatric Gliomas Recruiting NCT01837862 Phase 1, Phase 2 Mebendazole;Vincristine;Carboplatin;Temozolomide;Bevacizumab;Irinotecan
3 Intraarterial Infusion Of Erbitux and Bevacizumab For Relapsed/Refractory Intracranial Glioma In Patients Under 22 Recruiting NCT01884740 Phase 1, Phase 2 SIACI of Erbitux and Bevacizumab
4 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2 crizotinib
5 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
6 Imetelstat Sodium in Treating Young Patients With Refractory or Recurrent Solid Tumors or Lymphoma Completed NCT01273090 Phase 1 imetelstat sodium
7 18F-FDOPA PET/CT or PET/MRI in Measuring Tumors in Patients With Newly-Diagnosed or Recurrent Gliomas Terminated NCT02175745 Not Applicable 18F-fluoro-dihydroxyphenylalanine
8 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable

Search NIH Clinical Center for Pilomyxoid Astrocytoma

Genetic Tests for Pilomyxoid Astrocytoma

Anatomical Context for Pilomyxoid Astrocytoma

MalaCards organs/tissues related to Pilomyxoid Astrocytoma:

41
Brain, Spinal Cord, Cerebellum, Pineal, Hypothalamus

Publications for Pilomyxoid Astrocytoma

Articles related to Pilomyxoid Astrocytoma:

(show top 50) (show all 71)
# Title Authors Year
1
Intermediate Pilomyxoid Astrocytoma in the Cerebellum of a 5-Year-Old Boy. ( 29717569 )
2018
2
Fluorescence guided resection with 5-aminolevulinic acid of a pilomyxoid astrocytoma of the third ventricle. ( 28495088 )
2017
3
Bevacizumab Therapy for Pilomyxoid Astrocytoma. ( 28338567 )
2017
4
Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients. ( 28741227 )
2017
5
SOX10 Distinguishes Pilocytic and Pilomyxoid Astrocytomas From Ependymomas but Shows No Differences in Expression Level in Ependymomas From Infants Versus Older Children or Among Molecular Subgroups. ( 26945037 )
2016
6
Disseminated pilomyxoid astrocytoma in infancy with novel MUTYH mutation. ( 27797849 )
2016
7
Single-Agent Carboplatin for a Rare Case of Pilomyxoid Astrocytoma of the Spinal Cord in an Adult with Neurofibromatosis Type 1. ( 27920686 )
2016
8
Cytopathological features of pilomyxoid astrocytoma: a case report. ( 27018339 )
2016
9
Pilomyxoid astrocytoma in the adult cerebellum. ( 26777083 )
2016
10
Pilomyxoid astrocytoma involving the entire spinal cord in a newborn. ( 27106533 )
2016
11
Parietal pilomyxoid astrocytoma with recurrence in 10 months: A case report and review of literature. ( 27366287 )
2016
12
Spontaneous intratumoural and intraventricular haemorrhage associated with a pilomyxoid astrocytoma in the hypothalamic/chiasmatic region. ( 27450285 )
2016
13
Optic nerve pilomyxoid astrocytoma in a patient with Noonan syndrome. ( 25585602 )
2015
14
Cystic pilomyxoid astrocytoma on suprasellar region in 7-year-old girl: Treatment and strategy. ( 25972955 )
2015
15
Pilomyxoid Astrocytoma Occurring in the Third Ventricle. ( 26312139 )
2015
16
Pilomyxoid astrocytoma in an adult woman: Case report. ( 26458706 )
2015
17
Pilomyxoid Astrocytoma (PMA) Shows Significant Differences in Gene Expression vs. Pilocytic Astrocytoma (PA) and Variable Tendency Toward Maturation to PA. ( 25521223 )
2014
18
Pilomyxoid astrocytoma in an elderly patient: A case report and review of literature. ( 25387630 )
2014
19
Pilomyxoid astrocytoma presenting as diencephalic syndrome. ( 25672198 )
2014
20
Pilomyxoid astrocytoma treated successfully with vemurafenib. ( 24821190 )
2014
21
Pilomyxoid astrocytoma of the cerebellar vermis in an elderly patient. ( 24778917 )
2014
22
Pilomyxoid astrocytoma of the pineal region: Cytopathological features and differential diagnostic considerations by intraoperative smear preparation. ( 24578310 )
2014
23
Occurrence and distribution of pilomyxoid astrocytoma. ( 23281683 )
2013
24
Pediatric spinal pilomyxoid astrocytoma. ( 24053650 )
2013
25
Sustained response to weekly vinblastine in 2 children with pilomyxoid astrocytoma associated with diencephalic syndrome. ( 23042016 )
2013
26
Pilomyxoid astrocytoma of the cerebellum with Williams syndrome: a case report. ( 23604363 )
2013
27
Pilomyxoid astrocytoma of the brainstem. ( 23888217 )
2013
28
Primary pilomyxoid astrocytoma of the thoracolumbar spinal cord in an adult. ( 24441351 )
2013
29
Pilomyxoid astrocytoma with high proliferation index. ( 24470824 )
2013
30
Response. Pilomyxoid astrocytoma. ( 23767069 )
2013
31
Response. Pilomyxoid astrocytoma. ( 23767070 )
2013
32
Significance of radical resection for pilomyxoid astrocytoma of the cerebellum: a case report and review of the literature. ( 23503634 )
2013
33
Pilomyxoid astrocytoma. ( 23495811 )
2013
34
Insulin-like growth factor 2 mRNA binding protein 3 expression is an independent prognostic factor in pediatric pilocytic and pilomyxoid astrocytoma. ( 23584203 )
2013
35
Temporal and optic pathway pilomyxoid astrocytoma mimicking dural-based lesion: case report and review of the literature. ( 23548417 )
2012
36
Successful treatment of leptomeningeal gliomatosis of pilomyxoid astrocytoma after failed frontline chemotherapy. ( 22217612 )
2012
37
Intermediate pilomyxoid astrocytoma and diencephalic syndrome: imaging findings. ( 23052462 )
2012
38
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligoden-droglioma-like features. ( 22826787 )
2012
39
T2 and DWI in pilocytic and pilomyxoid astrocytoma with pathologic correlation. ( 22728857 )
2012
40
Pilomyxoid astrocytoma in cerebellum. ( 23467810 )
2011
41
Pilomyxoid astrocytoma of the cervical spinal cord in a child with rapid progression into glioblastoma: case report and literature review. ( 20461521 )
2011
42
Diencephalic juvenile pilomyxoid astrocytoma with leptomeningeal dissemination. ( 21534206 )
2011
43
Pilomyxoid astrocytoma of the cerebellum. ( 21529196 )
2011
44
Pilomyxoid astrocytoma in unusual location in a child with neurofibromatosis type 1: case report and review of the literature. ( 20496528 )
2010
45
Pilomyxoid astrocytoma. ( 20551536 )
2010
46
Pilomyxoid astrocytoma of the cervical spinal cord successfully treated with chemotherapy: case report. ( 21030812 )
2010
47
Juvenile pilomyxoid astrocytoma in the opticohypothalamus. ( 21286484 )
2010
48
Hemorrhagic onset of pilocytic astrocytoma and pilomyxoid astrocytoma. ( 19408090 )
2009
49
A case of pilomyxoid astrocytoma presenting with CSF rhinorrhoea in a 15-year-old. ( 19718549 )
2009
50
Pilomyxoid astrocytoma with involvement of the sella turcica in an adolescent. ( 19766001 )
2009

Variations for Pilomyxoid Astrocytoma

Copy number variations for Pilomyxoid Astrocytoma from CNVD:

7 (show all 14)
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 38162 10 1 3000000 Gain DIP2C Pilomyxoid astrocytoma
2 97080 15 98500000 102531392 Loss LASS3 Pilomyxoid astrocytoma
3 121279 18 41800000 46400000 Microdeletion TCF4 Pilomyxoid astrocytoma
4 234564 8 136400000 146364022 Gain COL22A1 Pilomyxoid astrocytoma
5 234874 8 139900000 146364022 Gain CYHR1 Pilomyxoid astrocytoma
6 234879 8 139900000 146364022 Gain FOXH1 Pilomyxoid astrocytoma
7 234882 8 139900000 146364022 Gain GPT Pilomyxoid astrocytoma
8 234885 8 139900000 146364022 Gain KIFC2 Pilomyxoid astrocytoma
9 234887 8 139900000 146364022 Gain LRRC14 Pilomyxoid astrocytoma
10 234889 8 139900000 146364022 Gain LRRC24 Pilomyxoid astrocytoma
11 234891 8 139900000 146364022 Gain MFSD3 Pilomyxoid astrocytoma
12 234894 8 139900000 146364022 Gain PPP1R16A Pilomyxoid astrocytoma
13 234898 8 139900000 146364022 Gain RECQL4 Pilomyxoid astrocytoma
14 244901 9 1 2200000 Loss ANKRD15 Pilomyxoid astrocytoma

Expression for Pilomyxoid Astrocytoma

Search GEO for disease gene expression data for Pilomyxoid Astrocytoma.

Pathways for Pilomyxoid Astrocytoma

Pathways related to Pilomyxoid Astrocytoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.85 GFAP SYP
2 10.41 COL1A1 COL2A1

GO Terms for Pilomyxoid Astrocytoma

Cellular components related to Pilomyxoid Astrocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 collagen trimer GO:0005581 8.62 COL1A1 COL2A1

Biological processes related to Pilomyxoid Astrocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ossification GO:0001503 9.46 COL1A1 COL2A1
2 collagen catabolic process GO:0030574 9.43 COL1A1 COL2A1
3 skin development GO:0043588 9.4 COL1A1 SUFU
4 collagen fibril organization GO:0030199 9.37 COL1A1 COL2A1
5 extracellular matrix organization GO:0030198 9.33 COL1A1 COL2A1 GFAP
6 skeletal system morphogenesis GO:0048705 9.32 COL1A1 COL2A1
7 endochondral ossification GO:0001958 9.26 COL1A1 COL2A1
8 skeletal system development GO:0001501 9.13 COL1A1 COL2A1 SUFU
9 cartilage development involved in endochondral bone morphogenesis GO:0060351 8.62 COL1A1 COL2A1

Molecular functions related to Pilomyxoid Astrocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 COL1A1 COL2A1 GFAP SYP
2 extracellular matrix structural constituent GO:0005201 9.16 COL1A1 COL2A1
3 platelet-derived growth factor binding GO:0048407 8.62 COL1A1 COL2A1

Sources for Pilomyxoid Astrocytoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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