MCID: MDL002
MIFTS: 44

Medulloepithelioma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Medulloepithelioma

MalaCards integrated aliases for Medulloepithelioma:

Name: Medulloepithelioma 12 15 73
Medulloepithelioma of the Central Nervous System 59
Medulloepithelioma, Central Nervous System 12
Diktyoma, Malignant 12

Characteristics:

Orphanet epidemiological data:

59
medulloepithelioma of the central nervous system
Inheritance: Not applicable; Age of onset: Childhood,Infancy;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4790
MeSH 44 D018242
NCIt 50 C4327 C66808
SNOMED-CT 68 39005004
Orphanet 59 ORPHA251883
UMLS via Orphanet 74 C0334596
UMLS 73 C0334596

Summaries for Medulloepithelioma

MalaCards based summary : Medulloepithelioma, also known as medulloepithelioma of the central nervous system, is related to ependymoblastoma and pineoblastoma, and has symptoms including vomiting, headache and nausea. An important gene associated with Medulloepithelioma is SYP (Synaptophysin), and among its related pathways/superpathways are Cytoskeletal Signaling and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Cisplatin and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and kidney.

Wikipedia : 76 Medulloepithelioma is a rare, primitive, fast-growing brain tumour thought to stem from cells of the... more...

Related Diseases for Medulloepithelioma

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

(show top 50) (show all 244)
# Related Disease Score Top Affiliating Genes
1 ependymoblastoma 30.1 GFAP NES VIM
2 pineoblastoma 30.0 GFAP SYP
3 ependymoma 29.6 GFAP NES SYP VIM
4 intraocular medulloepithelioma 12.3
5 optic nerve neoplasm 10.2 NES SYP
6 pleuropulmonary blastoma 10.2
7 infratentorial cancer 10.2 NES SYP
8 central nervous system benign neoplasm 10.2 GFAP SYP
9 nervous system benign neoplasm 10.2 GFAP SYP
10 atypical teratoid rhabdoid tumor 10.2 GFAP SYP
11 giant cell glioblastoma 10.2 GFAP SYP
12 cerebellopontine angle tumor 10.2 GFAP SYP
13 adult medulloblastoma 10.2 GFAP SYP
14 pilomyxoid astrocytoma 10.2 GFAP SYP
15 hemangioblastoma 10.2 GFAP SYP
16 cerebellar liponeurocytoma 10.2 GFAP SYP
17 retinitis pigmentosa 42 10.2 GFAP NES
18 brain glioma 10.2 GFAP NES
19 desmoplastic infantile ganglioglioma 10.2 GFAP SYP
20 extraventricular neurocytoma 10.2 GFAP SYP
21 lung combined type small cell carcinoma 10.2 GFAP SYP
22 alveolar soft part sarcoma 10.2 SYP VIM
23 rhabdoid tumor predisposition syndrome 1 10.2 SYP VIM
24 cellular ependymoma 10.2 GFAP SYP
25 ganglioneuroma 10.2 GFAP SYP
26 cerebral primitive neuroectodermal tumor 10.2 SYP VIM
27 anaplastic ganglioglioma 10.2 GFAP NES
28 dermatofibrosarcoma protuberans 10.2 NES VIM
29 chiari malformation 10.2 GFAP SYP
30 cerebral neuroblastoma 10.2 GFAP SYP
31 subependymal glioma 10.2 GFAP SYP
32 osteochondroma 10.2 NES VIM
33 neuronal migration disorders 10.2 NES VIM
34 central nervous system germ cell tumor 10.2 GFAP NES
35 embryonal tumor with multilayered rosettes 10.2
36 cerebral ventricle cancer 10.2 GFAP SYP
37 pineal gland cancer 10.1 GFAP SYP
38 primary hepatic neuroendocrine carcinoma 10.1 SYP VIM
39 meningioma, radiation-induced 10.1 GFAP VIM
40 papillary tumor of the pineal region 10.1 SYP VIM
41 benign ependymoma 10.1 GFAP SYP
42 myoepithelioma 10.1 GFAP VIM
43 obstructive hydrocephalus 10.1 GFAP SYP
44 pleomorphic adenoma 10.1 GFAP VIM
45 pineocytoma 10.1 GFAP SYP
46 tubular adenocarcinoma 10.1 KRT20 SYP
47 benign glioma 10.1 GFAP SYP
48 syringoma 10.1 GFAP VIM
49 primitive neuroectodermal tumor of the cervix uteri 10.1 GFAP VIM
50 cutaneous leiomyosarcoma 10.1 GFAP VIM

Graphical network of the top 20 diseases related to Medulloepithelioma:



Diseases related to Medulloepithelioma

Symptoms & Phenotypes for Medulloepithelioma

UMLS symptoms related to Medulloepithelioma:


vomiting, headache, nausea

Drugs & Therapeutics for Medulloepithelioma

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

(show top 50) (show all 84)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cisplatin Approved Phase 3 15663-27-1 2767 441203 84093
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3,Phase 1 1177-87-3
3
Ondansetron Approved Phase 3 99614-02-5 4595
4
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
5
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 1 50-02-2 5743
6 Gastrointestinal Agents Phase 3,Phase 1,Not Applicable
7 Antiemetics Phase 3,Phase 1
8 Autonomic Agents Phase 3,Phase 1
9 BB 1101 Phase 3,Phase 1
10 Peripheral Nervous System Agents Phase 3,Phase 1
11 Emetics Phase 3
12
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
13
Mechlorethamine Approved, Investigational Phase 1, Phase 2,Phase 2 51-75-2 4033
14
Carboplatin Approved Phase 1, Phase 2,Phase 2 41575-94-4 10339178 38904 498142
15
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
16
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
17
Melphalan Approved Phase 1, Phase 2 148-82-3 4053 460612
18
Mannitol Approved, Investigational Phase 1, Phase 2 69-65-8 453 6251
19 Alkylating Agents Phase 1, Phase 2,Phase 2
20 Antineoplastic Agents, Alkylating Phase 1, Phase 2,Phase 2
21 Anti-Bacterial Agents Phase 1, Phase 2
22 Anti-Infective Agents Phase 1, Phase 2
23 Immunologic Factors Phase 1, Phase 2
24 Immunosuppressive Agents Phase 1, Phase 2
25
Isophosphamide mustard Phase 1, Phase 2 0
26 Protein Kinase Inhibitors Phase 1, Phase 2
27 Liver Extracts Phase 1, Phase 2
28 Topoisomerase Inhibitors Phase 1, Phase 2
29 Etoposide phosphate Phase 1, Phase 2
30 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
31 Protective Agents Phase 1, Phase 2
32 alanine Phase 1, Phase 2
33 Antidotes Phase 1, Phase 2
34 diuretics Phase 1, Phase 2
35 phenylalanine Phase 1, Phase 2
36 Antioxidants Phase 1, Phase 2
37 Nitrogen Mustard Compounds Phase 1, Phase 2
38 Chelating Agents Phase 1, Phase 2
39 Antitubercular Agents Phase 1, Phase 2
40 Natriuretic Agents Phase 1, Phase 2
41 sodium thiosulfate Phase 1, Phase 2
42
Dacarbazine Approved, Investigational Phase 1 4342-03-4 5351166
43
Temozolomide Approved, Investigational Phase 1 85622-93-1 5394
44
Vorinostat Approved, Investigational Phase 1 149647-78-9 5311
45
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
46
Lenalidomide Approved Phase 1 191732-72-6 216326
47
Lenograstim Approved, Investigational Phase 1 135968-09-1
48
Gemcitabine Approved Phase 1 95058-81-4 60750
49
Trametinib Approved Phase 1 871700-17-3 11707110
50
Piperazine Approved, Vet_approved Phase 1 110-85-0 4837

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 Acupressure in Controlling Nausea in Young Patients Receiving Highly Emetogenic Chemotherapy Completed NCT01346267 Phase 3
2 Antineoplaston Therapy in Treating Children With Brain Tumors Completed NCT00003458 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
3 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
4 Melphalan, Carboplatin, Mannitol, and Sodium Thiosulfate in Treating Patients With Recurrent or Progressive CNS Embryonal or Germ Cell Tumors Suspended NCT00983398 Phase 1, Phase 2 Carboplatin;Mannitol;Melphalan;Sodium Thiosulfate
5 Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors Completed NCT01076530 Phase 1 vorinostat;temozolomide
6 Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma Completed NCT00994500 Phase 1 vorinostat;bortezomib
7 ABT-888 and Temozolomide in Treating Young Patients With Recurrent or Refractory CNS Tumors Completed NCT00946335 Phase 1 veliparib;temozolomide
8 Lenalidomide in Treating Young Patients With Recurrent, Progressive, or Refractory CNS Tumors Completed NCT00100880 Phase 1 lenalidomide
9 Cilengitide in Treating Children With Refractory Primary Brain Tumors Completed NCT00063973 Phase 1 cilengitide
10 Temozolomide and O6-Benzylguanine in Treating Children With Recurrent Brain Tumors Completed NCT00052780 Phase 1 O6-benzylguanine;temozolomide
11 PTC299 in Treating Young Patients With Refractory or Recurrent Primary Central Nervous System Tumors Completed NCT01158300 Phase 1 VEGF inhibitor PTC299
12 SJDAWN: St. Jude Children's Research Hospital Phase 1 Study Evaluating Molecularly-Driven Doublet Therapies for Children and Young Adults With Recurrent Brain Tumors Recruiting NCT03434262 Phase 1 Gemcitabine;ribociclib;sonidegib;trametinib
13 Palbociclib Isethionate in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors Recruiting NCT02255461 Phase 1 palbociclib isethionate
14 Gamma-Secretase Inhibitor RO4929097 in Treating Young Patients With Relapsed or Refractory Solid Tumors, CNS Tumors, Lymphoma, or T-Cell Leukemia Terminated NCT01088763 Phase 1 gamma-secretase/Notch signalling pathway inhibitor RO4929097;dexamethasone
15 Decision Making About Participating in Pharmacokinetic Studies in Patients Enrolled in a Phase I Treatment Clinical Trial Completed NCT00890435
16 DICER1-related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study Recruiting NCT01247597
17 Elucidating the Genetic Basis of the Pleuropulmonary Blastoma (PPB) Familial Cancer Syndrome Recruiting NCT00565903
18 International PPB Registry for PPB, DICER1 and Associated Conditions Recruiting NCT03382158
19 An Intermediate Expanded Use Trial of DFMO Available NCT03581240 eflornithine HCl
20 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable
21 Biomarkers in Blood Samples From Young Patients With Newly Diagnosed Brain Tumors Undergoing Standard Radiation Therapy and Chemotherapy Withdrawn NCT01233479 Not Applicable systemic chemotherapy

Search NIH Clinical Center for Medulloepithelioma

Genetic Tests for Medulloepithelioma

Anatomical Context for Medulloepithelioma

MalaCards organs/tissues related to Medulloepithelioma:

41
Brain, Eye, Kidney, Lung, Liver, Ovary, Thymus

Publications for Medulloepithelioma

Articles related to Medulloepithelioma:

(show top 50) (show all 179)
# Title Authors Year
1
Early Neuroblastic and Astrocytic Differentiation Demonstrated Immunohistochemically in a Small Intraocular Medulloepithelioma. ( 29765950 )
2018
2
Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports. ( 29768355 )
2018
3
Intraocular Medulloepithelioma: AIRP Best Cases in Radiologic-Pathologic Correlation. ( 29320332 )
2018
4
Intracranial medulloepithelioma in a child: a case report. ( 29757453 )
2018
5
Successful treatment of metastatic congenital intraocular medulloepithelioma with neoadjuvant chemotherapy, enucleation and superficial parotidectomy. ( 30128367 )
2018
6
A clinical case of Zellweger syndrome in a patient with a previous history of ocular medulloepithelioma. ( 30224891 )
2018
7
Conventional, diffusion, and permeability MR findings in ocular medulloepithelioma. ( 30232518 )
2018
8
Plaque Radiotherapy for Medulloepithelioma in 6 Cases From a Single Center. ( 30375203 )
2018
9
Combined morphological, immunohistochemical and genetic analyses of medulloepithelioma in the posterior cranial fossa. ( 28971535 )
2017
10
Posterior cerebral fossa medulloepithelioma: report of a case. ( 29200961 )
2017
11
Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report. ( 28217870 )
2017
12
Pigmented ciliary body medulloepithelioma in a newborn infant. ( 29118511 )
2017
13
Malignant Ciliary Body Medulloepithelioma With Brain and Parotid Metastasis. ( 28453163 )
2017
14
Intra-ocular medulloepithelioma as a masquerade for PHPV and Panophthalmitis: a Diagnostic Dilemma. ( 28559724 )
2017
15
Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis. ( 27254319 )
2016
16
Clinical behavior of intraocular teratoid medulloepithelioma in two-related Quarter Horses. ( 27440405 )
2016
17
Recurrent lacrimal gland choristoma of the ciliary body in an infant mimicking a medulloepithelioma. ( 27173894 )
2016
18
The Targetable Epigenetic Tumor Protein EZH2 is Enriched in Intraocular Medulloepithelioma. ( 27842164 )
2016
19
On the Classification and Grading of Medulloepithelioma of the Eye. ( 27239464 )
2016
20
Orbital medulloepithelioma in an adult patient: Radiation-induced second neoplasia? ( 27715363 )
2016
21
Nonteratoid Medulloepithelioma Presenting in a 78-Year-Old Male. ( 27843898 )
2016
22
Somatic DICER1 gene mutation in sporadic intraocular medulloepithelioma without pleuropulmonary blastoma syndrome. ( 25791583 )
2015
23
Medulloepithelioma with peculiar clinical presentation, stem cell phenotype and aberrant DNA-methylation profile. ( 26118758 )
2015
24
Ruthenium-106 Plaque Brachytherapy in the Primary Management of Ocular Medulloepithelioma. ( 25863421 )
2015
25
An Infant With Medulloepithelioma Successfully Treated by High-dose Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation Without Radiotherapy. ( 26181425 )
2015
26
Ciliary body medulloepithelioma association with pleuropulmonary blastoma in a familial tumor predisposition syndrome. ( 25032694 )
2014
27
Neovascular glaucoma in a child: an unusual presentation of medulloepithelioma. ( 27625938 )
2014
28
Unusual occurrence of supratentorial medulloepithelioma in a young female. ( 25002766 )
2014
29
Pigmented malignant teratoid medulloepithelioma of ciliary body - An extremely rare intraocular tumor in adult. ( 25278807 )
2014
30
New Insights Into the Development of Infantile Intraocular Medulloepithelioma. ( 25174896 )
2014
31
Peripheral medulloepithelioma: a rare tumor with a potential target therapy. ( 24559248 )
2014
32
Medulloepithelioma: A triad of clinical features. ( 25136238 )
2014
33
Primitive neuroectodermal tumour in a striped dolphin (Stenella coeruleoalba) with features of ependymoma and neural tube differentiation (Medulloepithelioma). ( 24011906 )
2013
34
Imaging features of medulloepithelioma: report of four cases and review of the literature. ( 23989751 )
2013
35
Malignant teratoid medulloepithelioma with retinoblastic and rhabdomyoblastic differentiation. ( 23791415 )
2013
36
Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. ( 24337497 )
2013
37
Ciliary body medulloepithelioma: analysis of 41 cases. ( 23796765 )
2013
38
Ciliary body medulloepithelioma associated with pleuropulmonary blastoma. ( 23613510 )
2013
39
Ciliary body medulloepithelioma in an adult. ( 23218809 )
2013
40
Malignant teratoid ciliary body medulloepithelioma in a neonate. ( 24261320 )
2013
41
Medulloepithelioma in DICER1 syndrome treated with resection. ( 23640610 )
2013
42
[Are the medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes the same entity?]. ( 24558755 )
2013
43
Benign intraocular teratoid medulloepithelioma causing glaucoma in an 11-year-old Arabian mare. ( 23025750 )
2013
44
Infratentorial medulloepithelioma with divergent differentiation: Possibly a predictor of poor outcome. ( 23248698 )
2012
45
Ciliary body medulloepithelioma in a 10-year-old boy. ( 22776925 )
2012
46
Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report. ( 22571440 )
2012
47
Intraocular medulloepithelioma. ( 22288972 )
2012
48
The masquerades of a childhood ciliary body medulloepithelioma: a case of chronic uveitis, cataract, and secondary glaucoma. ( 22606494 )
2012
49
Dilemma in management of ocular medulloepithelioma in a child. ( 22340579 )
2012
50
Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma--a report from the International Pleuropulmonary Blastoma Registry. ( 21156700 )
2011

Variations for Medulloepithelioma

Expression for Medulloepithelioma

Search GEO for disease gene expression data for Medulloepithelioma.

Pathways for Medulloepithelioma

Pathways related to Medulloepithelioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.82 KRT20 KRT7 VIM
2 11.15 GFAP NES SYP VIM
3
Show member pathways
11.05 GFAP INA KRT7 NES VIM

GO Terms for Medulloepithelioma

Cellular components related to Medulloepithelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neurofilament GO:0005883 9.16 GFAP INA
2 intermediate filament cytoskeleton GO:0045111 9.13 GFAP INA NES
3 intermediate filament GO:0005882 9.1 GFAP INA KRT20 KRT7 NES VIM

Biological processes related to Medulloepithelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of neuron projection development GO:0010977 9.32 GFAP VIM
2 astrocyte development GO:0014002 9.26 GFAP VIM
3 Bergmann glial cell differentiation GO:0060020 9.16 GFAP VIM
4 intermediate filament-based process GO:0045103 8.96 GFAP VIM
5 intermediate filament organization GO:0045109 8.8 GFAP KRT20 VIM

Molecular functions related to Medulloepithelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 9.26 GFAP KRT7 NES VIM
2 structural constituent of cytoskeleton GO:0005200 8.92 GFAP INA KRT20 VIM

Sources for Medulloepithelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
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30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
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44 MeSH
45 MESH via Orphanet
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49 NCI
50 NCIt
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54 NINDS
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58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
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74 UMLS via Orphanet
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