MCID: EPT008
MIFTS: 30

Epithelioid Malignant Peripheral Nerve Sheath Tumor malady

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Aliases & Descriptions 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 47 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 bronchial mucus gland adenoma. 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 are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Doxorubicin and Ifosfamide 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 127)
id Related Disease Score Top Affiliating Genes
1 malignant peripheral nerve sheath tumor 10.6
2 bronchial mucus gland adenoma 10.2 GFAP S100B
3 jejunal neuroendocrine tumor 10.2 CHGA S100B
4 adenofibroma 10.2 GFAP S100B
5 hypogonadotropic hypogonadism-retinitis pigmentosa syndrome 10.2 GFAP S100B
6 auricular cancer 10.2 GFAP S100B
7 thrombophlebitis 10.2 GFAP SMARCB1
8 skin glomus tumor 10.2 GFAP S100B
9 myxopapillary ependymoma 10.2 GFAP SMARCB1
10 ampulla of vater mucinous adenocarcinoma 10.2 CHGA S100B
11 intestinal pseudo-obstruction 10.2 GFAP SMARCB1
12 mast-cell sarcoma 10.2 GFAP SMARCB1
13 olfactory neuroblastoma 10.2 GFAP SMARCB1
14 pineoblastoma 10.2 GFAP SMARCB1
15 lucey-driscoll syndrome 10.2 MUC1 S100B
16 secondary pulmonary hemosiderosis 10.2 MUC1 S100B
17 invasive mole 10.2 MUC1 S100B
18 childhood choroid plexus cancer 10.2 GFAP SMARCB1
19 brain stem ependymoma 10.2 CHGA GFAP
20 myoma 10.2 MUC1 S100B
21 pemphigus vegetans 10.2 MUC1 S100B
22 postgerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma 10.2 MUC1 S100B
23 corneal intraepithelial neoplasm 10.2 MUC1 S100B
24 subglottis verrucous carcinoma 10.1 MUC1 S100B
25 pancreatic cancer 10.1 GFAP SMARCB1
26 conjunctival cancer 10.1 MUC1 S100B
27 clear cell ependymoma 10.1 MUC1 S100B
28 endocardium cancer 10.1 MUC1 S100B
29 urea cycle disorder 10.1 GFAP S100B
30 precursor t-lymphoblastic lymphoma/leukemia 10.1 MUC1 S100B
31 temporal lobe neoplasm 10.1 GFAP SMARCB1
32 mediastinum liposarcoma 10.1 MUC1 S100B
33 melanotic neurilemmoma 10.1 GFAP SMARCB1
34 superior vena cava syndrome 10.1 CHGA GFAP
35 calcific tendinitis 10.1 CHGA GFAP
36 pulmonary neuroendocrine tumor 10.1 MUC1 S100B
37 uterine corpus leiomyomatosis 10.1 MUC1 S100B
38 bone dysplasia azouz type 10.1 CHGA GFAP
39 gastrointestinal system cancer 10.1 MUC1 S100B
40 diarrhea, chronic, with villous atrophy 10.1 MUC1 S100B
41 prolactin producing pituitary tumor 10.1 CHGA MUC1
42 vagina leiomyosarcoma 10.1 GFAP MUC1
43 childhood brain germinoma 10.1 MUC1 S100B
44 desmoid tumor 10.1 MUC1 S100B
45 lung benign neoplasm 10.1 MUC1 SMARCB1
46 monostotic fibrous dysplasia 10.1 MUC1 S100B
47 childhood medulloblastoma 10.1 GFAP SMARCB1
48 breast secretory carcinoma 10.1 CHGA MUC1
49 popliteal pterygium syndrome, bartsocas-papas type 10.1 MUC1 S100B
50 myoepithelial carcinoma 10.1 CHGA GFAP

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 top 50) (show all 76)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 2,Phase 1 23214-92-8 31703
2
Ifosfamide Approved Phase 3,Phase 2,Phase 1 3778-73-2 3690
3
Mechlorethamine Approved Phase 3,Phase 2,Phase 1 51-75-2 4033
4
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
5
Etoposide Approved Phase 3 33419-42-0 36462
6
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
7
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
8
Lenograstim Approved Phase 3 135968-09-1
9
Dactinomycin Approved Phase 3 50-76-0 2019 457193
10
Epirubicin Approved Phase 3 56420-45-2 41867
11
Trabectedin Approved, Investigational Phase 2, Phase 3 114899-77-3 108150
12
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
13
Cyproheptadine Approved Phase 3 129-03-3 2913
14
Doxil Approved June 1999 Phase 3,Phase 2,Phase 1 31703
15 Alkylating Agents Phase 3,Phase 2,Phase 1
16 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1
17 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
18 Isophosphamide mustard Phase 3,Phase 2,Phase 1
19 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
20 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1
21 Nucleic Acid Synthesis Inhibitors Phase 3
22 Etoposide phosphate Phase 3
23 Anti-Infective Agents Phase 3,Phase 2
24 Immunosuppressive Agents Phase 3,Phase 2
25 Antimitotic Agents Phase 3,Phase 2
26 Antirheumatic Agents Phase 3
27 Antineoplastic Agents, Phytogenic Phase 3
28
Histamine Phosphate Phase 3 51-74-1 65513
29
Serotonin Phase 3 50-67-9 5202
30 Serotonin Agents Phase 3
31 Serotonin Antagonists Phase 3
32 Gastrointestinal Agents Phase 3
33 Neurotransmitter Agents Phase 3
34 Histamine Antagonists Phase 3
35 Histamine H1 Antagonists Phase 3
36 Dermatologic Agents Phase 3
37 Anti-Allergic Agents Phase 3
38 Antipruritics Phase 3
39
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
40
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
41
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
42
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
43
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
44
Gemcitabine Approved Phase 2 95058-81-4 60750
45
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
46
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
47
Everolimus Approved Phase 2 159351-69-6 6442177
48
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
49
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
50
Nicotinamide Approved, Nutraceutical Phase 2 98-92-0 936

Interventional clinical trials:

(show all 33)
id Name Status NCT ID Phase
1 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Unknown status NCT00346164 Phase 3
2 Ifosfamide and Doxorubicin, Radiation Therapy, and/or Surgery in Treating Young Patients With Localized Soft Tissue Sarcoma Unknown status NCT00334854 Phase 3
3 Doxorubicin With or Without Ifosfamide and Pegfilgrastim in Treating Patients With Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00061984 Phase 3
4 Surgery With or Without Radiation Therapy in Treating Patients With Primary Soft Tissue Sarcoma of the Retroperitoneum or Pelvis Completed NCT00091351 Phase 3
5 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3
6 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3
7 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
8 Randomised Trial of Volume of Post-operative Radiotherapy Given to Adult Patients With eXtremity Soft Tissue Sarcoma Active, not recruiting NCT00423618 Phase 3
9 Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3
10 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3
11 Temozolomide in Treating Patients With Advanced Soft Tissue Sarcoma Unknown status NCT00003718 Phase 2
12 Brostallicin or Doxorubicin as First-Line Therapy in Treating Patients With Relapsed, Refractory, or Metastatic Soft Tissue Sarcoma Unknown status NCT00410462 Phase 2
13 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2
14 Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma Completed NCT00245102 Phase 2
15 Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma Completed NCT00112463 Phase 2
16 STI571 in Treating Patients With Recurrent or Refractory Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2
17 Ecteinascidin 743 in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00003939 Phase 2
18 BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors Completed NCT00526149 Phase 2
19 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2
20 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
21 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2
22 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2
23 Trial of Dasatinib in Advanced Sarcomas Active, not recruiting NCT00464620 Phase 2
24 Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma Active, not recruiting NCT01614795 Phase 2
25 Intensity-Modulated Radiation Therapy in Treating Patients Undergoing Surgery for Stage IB, Stage II, or Stage III Soft Tissue Sarcoma Terminated NCT00740597 Phase 2
26 Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma Completed NCT00720174 Phase 1
27 Liposomal Doxorubicin Plus Ifosfamide in Treating Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00030784 Phase 1
28 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
29 Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery Recruiting NCT03009201 Phase 1
30 Tissue Sample Collection From Patients With Soft Tissue Sarcoma of the Arms, Hands, Legs, or Feet Treated on Clinial Trial CRUK-VORTEX Unknown status NCT00900211
31 Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas Completed NCT01567046
32 PET CT as Predictor of Response in Preoperative Chemotherapy for Soft Tissue Sarcoma Active, not recruiting NCT00346125
33 Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma Withdrawn NCT00334984

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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:

39
Thyroid

Publications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Articles related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

(show all 18)
id Title Authors Year
1
Fine-needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature. ( 26646298 )
2016
2
Huge epithelioid malignant peripheral nerve sheath tumor in the left axilla: a case report. ( 26366361 )
2015
3
A misdiagnosed melanoma: a case of cutaneous epithelioid malignant peripheral nerve sheath tumor. ( 25933073 )
2015
4
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. ( 25602794 )
2015
5
Rapidly progressive epithelioid malignant peripheral nerve sheath tumor of the vestibular nerve. ( 23988994 )
2013
6
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
7
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. ( 20952276 )
2011
8
Advanced epithelioid malignant peripheral nerve sheath tumor showing complete response to combined surgery and chemotherapy: a case report. ( 22606446 )
2011
9
Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease. ( 17972308 )
2008
10
Epithelioid malignant peripheral nerve sheath tumor in the infraorbital nerve. ( 16550070 )
2006
11
Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus. ( 16382783 )
2005
12
Epithelioid malignant peripheral nerve sheath tumor. Report of a case with inflammatory infiltration. ( 15991844 )
2005
13
Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases. ( 15592781 )
2004
14
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
15
Intraosseous epithelioid malignant peripheral nerve sheath tumor of the phalanx. Case report. ( 10230694 )
1999
16
Purely epithelioid malignant peripheral nerve sheath tumor of the vulva. ( 9142667 )
1997
17
Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). ( 9285192 )
1997
18
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:

id Super pathways Score Top Affiliating Genes
1 11.31 GFAP NF1 S100B
2 10.86 GFAP 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:

id 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:

id 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
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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