MCID: EPT008
MIFTS: 29

Epithelioid Malignant Peripheral Nerve Sheath Tumor

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards integrated aliases for Epithelioid Malignant Peripheral Nerve Sheath Tumor:

Name: Epithelioid Malignant Peripheral Nerve Sheath Tumor 12 14 69
Malignant Epithelioid Neoplasm of the Peripheral Nerve Sheath 12
Epithelioid Mpnst 12

Classifications:



External Ids:

Disease Ontology 12 DOID:8353
NCIt 46 C6561
UMLS 69 C1321427

Summaries for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards based summary : Epithelioid Malignant Peripheral Nerve Sheath Tumor, also known as malignant epithelioid neoplasm of the peripheral nerve sheath, is related to malignant peripheral nerve sheath tumor and malignant triton tumor. An important gene associated with Epithelioid Malignant Peripheral Nerve Sheath Tumor is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1), and among its related pathways/superpathways is Neuroscience. The drugs Doxorubicin and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include thyroid.

Related Diseases for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Diseases related to Epithelioid Malignant Peripheral Nerve Sheath Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 166)
# Related Disease Score Top Affiliating Genes
1 malignant peripheral nerve sheath tumor 30.3 MUC1 NF1 S100B SMARCB1
2 malignant triton tumor 10.3 NF1 S100B
3 primary hepatic neuroendocrine carcinoma 10.3 CHGA S100B
4 pleomorphic xanthoastrocytoma 10.2 CHGA SMARCB1
5 localized hypertrophic neuropathy 10.2 MUC1 S100B
6 sclerosing perineurioma 10.2 MUC1 S100B
7 tanycytic ependymoma 10.2 CHGA SMARCB1
8 pancreatic gastrinoma 10.2 CHGA S100B
9 reticular perineurioma 10.2 MUC1 S100B
10 non-functioning pituitary adenoma 10.2 MUC1 S100B
11 cellular schwannoma 10.2 NF1 S100B
12 fibrous meningioma 10.2 MUC1 S100B
13 myxofibrosarcoma 10.2 MUC1 S100B
14 syringocystadenoma papilliferum 10.2 MUC1 S100B
15 eccrine porocarcinoma 10.2 MUC1 S100B
16 myoepithelioma 10.2 S100B SMARCB1
17 aggressive digital papillary adenocarcinoma 10.2 MUC1 S100B
18 neurilemmomatosis 10.2 NF1 SMARCB1
19 chordoid glioma 10.2 MUC1 S100B
20 myxopapillary ependymoma 10.2 MUC1 S100B
21 monophasic synovial sarcoma 10.2 MUC1 S100B
22 syringoma 10.2 MUC1 S100B
23 anaplastic ependymoma 10.2 MUC1 S100B
24 pleomorphic liposarcoma 10.2 MUC1 S100B
25 spindle cell sarcoma 10.2 MUC1 S100B
26 perineurioma 10.2 MUC1 S100B
27 spiradenoma 10.2 MUC1 S100B
28 papilloma of choroid plexus 10.2 MUC1 S100B
29 functioning pituitary adenoma 10.2 MUC1 S100B
30 dendritic cell tumor 10.2 MUC1 S100B
31 brain germinoma 10.2 NF1 SMARCB1
32 hemangiopericytoma, malignant 10.2 MUC1 S100B
33 polymorphous low-grade adenocarcinoma 10.1 MUC1 S100B
34 papillary adenocarcinoma 10.1 MUC1 S100B
35 clear cell meningioma 10.1 CHGA MUC1
36 malignant fibroxanthoma 10.1 MUC1 S100B
37 histiocytic sarcoma 10.1 MUC1 S100B
38 dermoid cyst 10.1 CHGA MUC1
39 ependymoma 10.1 MUC1 S100B
40 phaeochromocytoma 10.1 CHGA NF1
41 gastric tubular adenocarcinoma 10.1 CHGA MUC1
42 bednar tumor 10.1 ENO2 S100B
43 epithelioid sarcoma 10.1 MUC1 SMARCB1
44 chordoma 10.1 MUC1 S100B
45 middle cerebral artery infarction 10.1 ENO2 S100B
46 signet ring cell adenocarcinoma 10.1 CHGA MUC1
47 tubular adenocarcinoma 10.1 CHGA MUC1
48 head injury 10.1 ENO2 S100B
49 osteofibrous dysplasia 10.0 MUC1 NF1
50 large cell carcinoma with rhabdoid phenotype 10.0 ENO2 SMARCB1

Graphical network of the top 20 diseases related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:



Diseases related to Epithelioid Malignant Peripheral Nerve Sheath Tumor

Symptoms & Phenotypes for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Drugs & Therapeutics for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Drugs for Epithelioid Malignant Peripheral Nerve Sheath Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6 Isophosphamide mustard Phase 2, Phase 3 0
7 Alkylating Agents Phase 2, Phase 3
8 Topoisomerase Inhibitors Phase 2, Phase 3
9 Anti-Bacterial Agents Phase 2, Phase 3
10 Antibiotics, Antitubercular Phase 2, Phase 3
11 lysine Nutraceutical Phase 2,Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
2 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2 Tazemetostat
3 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Recruiting NCT02601937 Phase 1 Tazemetostat

Search NIH Clinical Center for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Genetic Tests for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Anatomical Context for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards organs/tissues related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

38
Thyroid

Publications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Articles related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

(show all 19)
# Title Authors Year
1
Metastatic Epithelioid Malignant Peripheral Nerve Sheath Tumor in a Known Case of Neurofibromatosis-1, Cytomorphological Appearance, and Critical Analysis of Immunohistochemistry. ( 29200699 )
2017
2
Fine-needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature. ( 26646298 )
2016
3
Huge epithelioid malignant peripheral nerve sheath tumor in the left axilla: a case report. ( 26366361 )
2015
4
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. ( 25602794 )
2015
5
A misdiagnosed melanoma: a case of cutaneous epithelioid malignant peripheral nerve sheath tumor. ( 25933073 )
2015
6
Rapidly progressive epithelioid malignant peripheral nerve sheath tumor of the vestibular nerve. ( 23988994 )
2013
7
Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with "neuroblastoma-like" schwannomatosis and a novel germline SMARCB1 mutation. ( 22082606 )
2012
8
Advanced epithelioid malignant peripheral nerve sheath tumor showing complete response to combined surgery and chemotherapy: a case report. ( 22606446 )
2011
9
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. ( 20952276 )
2011
10
Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease. ( 17972308 )
2008
11
Epithelioid malignant peripheral nerve sheath tumor in the infraorbital nerve. ( 16550070 )
2006
12
Epithelioid malignant peripheral nerve sheath tumor. Report of a case with inflammatory infiltration. ( 15991844 )
2005
13
Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus. ( 16382783 )
2005
14
Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases. ( 15592781 )
2004
15
Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. ( 11145248 )
2001
16
Intraosseous epithelioid malignant peripheral nerve sheath tumor of the phalanx. Case report. ( 10230694 )
1999
17
Purely epithelioid malignant peripheral nerve sheath tumor of the vulva. ( 9142667 )
1997
18
Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). ( 9285192 )
1997
19
The purely epithelioid malignant peripheral nerve sheath tumor. ( 2425646 )
1986

Variations for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Expression for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Search GEO for disease gene expression data for Epithelioid Malignant Peripheral Nerve Sheath Tumor.

Pathways for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Pathways related to Epithelioid Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.33 ENO2 NF1 S100B

GO Terms for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Biological processes related to Epithelioid Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of histone H4 acetylation GO:0090240 8.62 MUC1 SMARCB1

Molecular functions related to Epithelioid Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 p53 binding GO:0002039 8.62 MUC1 SMARCB1

Sources for Epithelioid Malignant Peripheral Nerve Sheath Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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