MCID: MDL002
MIFTS: 43

Medulloepithelioma malady

Categories: Neuronal diseases, Rare diseases, Cancer diseases

Aliases & Classifications for Medulloepithelioma

Aliases & Descriptions for Medulloepithelioma:

Name: Medulloepithelioma 12 14 69
Medulloepithelioma of the Central Nervous System 56
Medulloepithelioma, Central Nervous System 12
Medulloepithelioma Nos 12
Diktyoma, Malignant 12

Characteristics:

Orphanet epidemiological data:

56
medulloepithelioma of the central nervous system
Inheritance: Not applicable; Age of onset: Childhood,Infancy;

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4790
MeSH 42 D018242
NCIt 47 C4327 C66808
SNOMED-CT 64 189932005 39005004
Orphanet 56 ORPHA251883
UMLS via Orphanet 70 C0334596
UMLS 69 C0334596

Summaries for Medulloepithelioma

MalaCards based summary : Medulloepithelioma, also known as medulloepithelioma of the central nervous system, is related to pineoblastoma and intraocular medulloepithelioma, and has symptoms including headache, nausea and vomiting. An important gene associated with Medulloepithelioma is SYP (Synaptophysin), and among its related pathways/superpathways are Cytoskeletal Signaling and Striated Muscle Contraction. The drugs Dexamethasone and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include eye, brain and retina.

Wikipedia : 71 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 191)
id Related Disease Score Top Affiliating Genes
1 pineoblastoma 29.8 GFAP SYP
2 intraocular medulloepithelioma 12.0
3 diabetes hypogonadism deafness mental retardation 10.3 GFAP SYP
4 papillary tumor of the pineal region 10.2 GFAP SYP
5 brain stem ependymoma 10.2 GFAP SYP
6 intrahepatic bile duct adenoma 10.2 GFAP SYP
7 pilocytic astrocytoma of cerebellum 10.2 GFAP SYP
8 children's interstitial lung disease 10.2 GFAP SYP
9 cervical benign neoplasm 10.2 GFAP SYP
10 epidermal appendage tumor 10.2 GFAP SYP
11 clear cell meningioma 10.2 GFAP SYP
12 cerebral folate receptor alpha deficiency 10.2 GFAP SYP
13 pilocytic astrocytoma 10.2 GFAP SYP
14 hypothalamic neoplasm 10.2 GFAP NES
15 angiofollicular lymph hyperplasia 10.2 GFAP NES
16 blood group incompatibility 10.2 GFAP SYP
17 pleomorphic xanthoastrocytoma 10.2 GFAP SYP
18 mediastinal granular cell myoblastoma 10.2 GFAP SYP
19 central neurocytoma 10.2 GFAP SYP
20 mast-cell sarcoma 10.2 GFAP SYP
21 olfactory neuroblastoma 10.2 GFAP SYP
22 childhood kidney cell carcinoma 10.2 GFAP NES
23 piussan lenaerts mathieu syndrome 10.2 GFAP SYP
24 pneumothorax 10.2 GFAP SYP
25 brain stem astrocytic neoplasm 10.2 GFAP SYP
26 brain oligodendroglioma 10.2 GFAP SYP
27 arthus reaction 10.2 GFAP SYP
28 bone deterioration disease 10.2 DES SYP
29 osteopetrosis, autosomal recessive 8 10.2 GFAP NES
30 childhood central nervous system primitive neuroectodermal neoplasm 10.2 GFAP SYP
31 bone dysplasia azouz type 10.2 GFAP SYP
32 bronchiolitis obliterans 10.2 GFAP NES
33 pancreatic cancer 10.2 GFAP SYP
34 exudative glomerulonephritis 10.2 DES SYP
35 temporal lobe neoplasm 10.2 GFAP SYP
36 melanotic neurilemmoma 10.2 GFAP SYP
37 glucosidase acid-1,4-alpha deficiency 10.2 DES GFAP
38 clear cell variant infiltrating bladder urothelial carcinoma 10.2 GFAP SYP
39 vagina leiomyosarcoma 10.2 DES GFAP
40 granulomatous myositis 10.2 NES SYP
41 ovarian cystadenoma 10.2 DES GFAP
42 non specific chronic endometritis 10.2 KRT20 SYP
43 spasmodic dysphonia 10.2 KRT20 SYP
44 myoepithelial carcinoma 10.2 GFAP SYP
45 fallopian tube benign neoplasm 10.2 GFAP NES
46 pharynx cancer 10.2 GFAP SYP
47 ovarian cancer 10.2 GFAP INA
48 focal cortical dysplasia, taylor balloon cell type 10.2 GFAP SYP
49 paraphimosis 10.2 KRT20 SYP
50 pelvic inflammatory disease 10.2 KRT20 SYP

Graphical network of the top 20 diseases related to Medulloepithelioma:



Diseases related to Medulloepithelioma

Symptoms & Phenotypes for Medulloepithelioma

UMLS symptoms related to Medulloepithelioma:


headache, nausea, vomiting

Drugs & Therapeutics for Medulloepithelioma

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

(show top 50) (show all 68)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 1 50-02-2 5743
2
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
3
Ondansetron Approved Phase 3 99614-02-5 4595
4
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
5 Peripheral Nervous System Agents Phase 3,Phase 1
6 Dexamethasone acetate Phase 3,Phase 1 1177-87-3
7 Gastrointestinal Agents Phase 3,Phase 1
8 Dexamethasone 21-phosphate Phase 3,Phase 1
9 Emetics Phase 3
10 Antiemetics Phase 3,Phase 1
11 BB 1101 Phase 3,Phase 1
12 Autonomic Agents Phase 3,Phase 1
13
Mannitol Approved, Investigational Phase 1, Phase 2 69-65-8 453 6251
14
Melphalan Approved Phase 1, Phase 2 148-82-3 4053 460612
15
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
16
Mechlorethamine Approved Phase 1, Phase 2 51-75-2 4033
17 Sodium thiosulfate Approved Phase 1, Phase 2
18
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
19
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
20
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
21
Crizotinib Approved Phase 1, Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
22 Alkylating Agents Phase 1, Phase 2
23 Anti-Bacterial Agents Phase 1, Phase 2
24 Anti-Infective Agents Phase 1, Phase 2
25 Immunosuppressive Agents Phase 1, Phase 2
26 Antineoplastic Agents, Alkylating Phase 1, Phase 2
27 diuretics Phase 1, Phase 2
28 Chelating Agents Phase 1, Phase 2
29 Natriuretic Agents Phase 1, Phase 2
30 Nitrogen Mustard Compounds Phase 1, Phase 2
31 Protective Agents Phase 1, Phase 2
32 Antidotes Phase 1, Phase 2
33 Protein Kinase Inhibitors Phase 1, Phase 2
34 Antitubercular Agents Phase 1, Phase 2
35 Antioxidants Phase 1, Phase 2
36 Topoisomerase Inhibitors Phase 1, Phase 2
37 Etoposide phosphate Phase 1, Phase 2
38 Isophosphamide mustard Phase 1, Phase 2
39 Liver Extracts Phase 1, Phase 2
40 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
41 alanine Nutraceutical Phase 1, Phase 2
42 phenylalanine Nutraceutical Phase 1, Phase 2
43
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
44
Dacarbazine Approved, Investigational Phase 1 4342-03-4 5351166
45
Temozolomide Approved, Investigational Phase 1 85622-93-1 5394
46
Lenograstim Approved Phase 1 135968-09-1
47
Lenalidomide Approved Phase 1 191732-72-6 216326
48
Vorinostat Approved, Investigational Phase 1 149647-78-9 5311
49
Palbociclib Approved Phase 1 571190-30-2 11431660 5005498 5330286
50
Veliparib Investigational Phase 1 912444-00-9 11960529

Interventional clinical trials:

(show all 19)
id Name Status NCT ID Phase
1 Acupressure in Controlling Nausea in Young Patients Receiving Highly Emetogenic Chemotherapy Active, not recruiting NCT01346267 Phase 3
2 Antineoplaston Therapy in Treating Children With Brain Tumors Completed NCT00003458 Phase 2
3 Melphalan, Carboplatin, Mannitol, and Sodium Thiosulfate in Treating Patients With Recurrent or Progressive CNS Embryonal or Germ Cell Tumors Recruiting NCT00983398 Phase 1, Phase 2
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
5 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2
6 Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors Completed NCT01076530 Phase 1
7 Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma Completed NCT00994500 Phase 1
8 ABT-888 and Temozolomide in Treating Young Patients With Recurrent or Refractory CNS Tumors Completed NCT00946335 Phase 1
9 Lenalidomide in Treating Young Patients With Recurrent, Progressive, or Refractory CNS Tumors Completed NCT00100880 Phase 1
10 Cilengitide in Treating Children With Refractory Primary Brain Tumors Completed NCT00063973 Phase 1
11 Temozolomide and O6-Benzylguanine in Treating Children With Recurrent Brain Tumors Completed NCT00052780 Phase 1
12 PTC299 in Treating Young Patients With Refractory or Recurrent Primary Central Nervous System Tumors Completed NCT01158300 Phase 1
13 Palbociclib Isethionate in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors Recruiting NCT02255461 Phase 1
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
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 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452
19 Biomarkers in Blood Samples From Young Patients With Newly Diagnosed Brain Tumors Undergoing Standard Radiation Therapy and Chemotherapy Withdrawn NCT01233479

Search NIH Clinical Center for Medulloepithelioma

Genetic Tests for Medulloepithelioma

Anatomical Context for Medulloepithelioma

MalaCards organs/tissues related to Medulloepithelioma:

39
Eye, Brain, Retina, Bone, Kidney, Spinal Cord, Pons

Publications for Medulloepithelioma

Articles related to Medulloepithelioma:

(show top 50) (show all 165)
id Title Authors Year
1
On the Classification and Grading of Medulloepithelioma of the Eye. ( 27239464 )
2016
2
Somatic mutations of DICER1 and KMT2D are frequent in intraocular medulloepitheliomas. ( 26841698 )
2016
3
Clinical behavior of intraocular teratoid medulloepithelioma in two-related Quarter Horses. ( 27440405 )
2016
4
Intraocular Teratoid Medulloepithelioma Presenting With a Completely Rhabdomyosarcomatous Distant Metastasis. ( 27254319 )
2016
5
Recurrent lacrimal gland choristoma of the ciliary body in an infant mimicking a medulloepithelioma. ( 27173894 )
2016
6
The Targetable Epigenetic Tumor Protein EZH2 is Enriched in Intraocular Medulloepithelioma. ( 27842164 )
2016
7
Orbital medulloepithelioma in an adult patient: Radiation-induced second neoplasia? ( 27715363 )
2016
8
Nonteratoid Medulloepithelioma Presenting in a 78-Year-Old Male. ( 27843898 )
2016
9
Somatic DICER1 gene mutation in sporadic intraocular medulloepithelioma without pleuropulmonary blastoma syndrome. ( 25791583 )
2015
10
An Infant With Medulloepithelioma Successfully Treated by High-dose Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation Without Radiotherapy. ( 26181425 )
2015
11
Medulloepithelioma with peculiar clinical presentation, stem cell phenotype and aberrant DNA-methylation profile. ( 26118758 )
2015
12
Ruthenium-106 Plaque Brachytherapy in the Primary Management of Ocular Medulloepithelioma. ( 25863421 )
2015
13
Intraocular Medulloepitheliomas and Embryonal Tumors With Multilayered Rosettes of the Brain: Comparative Roles of LIN28A and C19MC. ( 25748578 )
2015
14
Pigmented malignant teratoid medulloepithelioma of ciliary body - An extremely rare intraocular tumor in adult. ( 25278807 )
2014
15
Ciliary body medulloepithelioma association with pleuropulmonary blastoma in a familial tumor predisposition syndrome. ( 25032694 )
2014
16
Peripheral medulloepithelioma: a rare tumor with a potential target therapy. ( 24559248 )
2014
17
Neovascular glaucoma in a child: an unusual presentation of medulloepithelioma. ( 27625938 )
2014
18
Medulloepithelioma: A triad of clinical features. ( 25136238 )
2014
19
Unusual occurrence of supratentorial medulloepithelioma in a young female. ( 25002766 )
2014
20
New Insights Into the Development of Infantile Intraocular Medulloepithelioma. ( 25174896 )
2014
21
Medulloepithelioma in DICER1 syndrome treated with resection. ( 23640610 )
2013
22
Malignant teratoid ciliary body medulloepithelioma in a neonate. ( 24261320 )
2013
23
Primitive neuroectodermal tumour in a striped dolphin (Stenella coeruleoalba) with features of ependymoma and neural tube differentiation (Medulloepithelioma). ( 24011906 )
2013
24
Ciliary body medulloepithelioma in an adult. ( 23218809 )
2013
25
Ciliary body medulloepithelioma: analysis of 41 cases. ( 23796765 )
2013
26
Ciliary body medulloepithelioma associated with pleuropulmonary blastoma. ( 23613510 )
2013
27
Benign intraocular teratoid medulloepithelioma causing glaucoma in an 11-year-old Arabian mare. ( 23025750 )
2013
28
Malignant teratoid medulloepithelioma with retinoblastic and rhabdomyoblastic differentiation. ( 23791415 )
2013
29
Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. ( 24337497 )
2013
30
Imaging features of medulloepithelioma: report of four cases and review of the literature. ( 23989751 )
2013
31
Dilemma in management of ocular medulloepithelioma in a child. ( 22340579 )
2012
32
Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report. ( 22571440 )
2012
33
Ciliary body medulloepithelioma in a 10-year-old boy. ( 22776925 )
2012
34
Infratentorial medulloepithelioma with divergent differentiation: Possibly a predictor of poor outcome. ( 23248698 )
2012
35
The masquerades of a childhood ciliary body medulloepithelioma: a case of chronic uveitis, cataract, and secondary glaucoma. ( 22606494 )
2012
36
Intraocular medulloepithelioma. ( 22288972 )
2012
37
Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement. ( 21995988 )
2011
38
Chemosensitivity of medulloepithelioma. ( 21724057 )
2011
39
Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Report of two cases treated according to the GPHO Protocol HIT 2000 and review of the literature. ( 22037651 )
2011
40
Ciliary body medulloepithelioma with neoplastic cyclitic membrane imaging with fluorescein angiography and ultrasound biomicroscopy. ( 21505878 )
2011
41
Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma--a report from the International Pleuropulmonary Blastoma Registry. ( 21156700 )
2011
42
Medulloepithelioma of the optic disc. ( 21676432 )
2011
43
Fine needle aspiration cytology of metastatic ocular medulloepithelioma. ( 21062379 )
2011
44
Ocular medulloepithelioma chemosensitivity. ( 20947172 )
2010
45
Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study. ( 19669095 )
2010
46
Intraoperative cytodiagnosis of ciliary body medulloepithelioma masquerading as anterior ciliary staphyloma. ( 20968164 )
2010
47
Malignant transformation of a medulloepithelioma of the optic nerve. ( 20497085 )
2010
48
Malignant non-teratoid medulloepithelioma of the optic nerve with intraocular extension. ( 20497433 )
2010
49
Conservative surgical treatment of medulloepithelioma of the ciliary body. ( 20212214 )
2010
50
Embryonal tumor with abundant neuropil and true rosettes: morphological, immunohistochemical, ultrastructural and molecular study of a case showing features of medulloepithelioma and areas of mesenchymal and epithelial differentiation. ( 19563506 )
2010

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:

id Super pathways Score Top Affiliating Genes
1 11.81 DES KRT20 VIM
2 11.18 DES VIM
3 11.16 GFAP NES SYP VIM
4
Show member pathways
11.05 DES GFAP INA NES VIM
5
Show member pathways
10.91 DES VIM

GO Terms for Medulloepithelioma

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

id Name GO ID Score Top Affiliating Genes
1 intermediate filament cytoskeleton GO:0045111 9.26 DES GFAP INA NES
2 neuromuscular junction GO:0031594 9.16 DES SYP
3 intermediate filament GO:0005882 9.1 DES GFAP INA KRT20 NES VIM

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

id Name GO ID Score Top Affiliating Genes
1 muscle filament sliding GO:0030049 8.96 DES VIM
2 intermediate filament organization GO:0045109 8.8 DES KRT20 VIM

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

id Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 DES GFAP SYP VIM
2 structural constituent of cytoskeleton GO:0005200 9.02 DES GFAP INA KRT20 VIM

Sources for Medulloepithelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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