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 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 spleen cancer. 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 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 161)
id Related Disease Score Top Affiliating Genes
1 malignant peripheral nerve sheath tumor 10.6
2 spleen cancer 10.5 NF1 S100B
3 neurogenic thoracic outlet syndrome 10.5 CHGA S100B
4 logopenic progressive aphasia 10.4 MUC1 S100B
5 heiner syndrome 10.4 MUC1 S100B
6 dedifferentiated liposarcoma 10.4 MUC1 S100B
7 c-p angle neurinoma 10.4 NF1 S100B
8 brain stem ependymoma 10.4 CHGA SMARCB1
9 simple cryoglobulinemia 10.4 MUC1 S100B
10 volkmann contracture 10.4 CHGA S100B
11 spleen angiosarcoma 10.4 MUC1 S100B
12 mucinous ovarian cystadenoma 10.4 MUC1 S100B
13 gliofibroma 10.4 CHGA SMARCB1
14 noninvasive malignant thymoma 10.4 MUC1 S100B
15 pancreatic intraductal papillary-colloid carcinoma 10.3 MUC1 S100B
16 lice infestation 10.3 MUC1 S100B
17 gastric papillary adenocarcinoma 10.3 MUC1 S100B
18 pleural empyema 10.3 MUC1 S100B
19 ganglioglioma 10.3 MUC1 S100B
20 anaplastic oligodendroglioma 10.3 MUC1 S100B
21 vulvar squamous papilloma 10.3 MUC1 S100B
22 spindle cell liposarcoma 10.3 MUC1 S100B
23 coronary restenosis 10.3 MUC1 S100B
24 pulmonary venoocclusive disease 10.3 MUC1 S100B
25 prostate disease 10.3 MUC1 S100B
26 strabismus 10.3 MUC1 S100B
27 dense deposit disease 10.3 MUC1 S100B
28 central nervous system sarcoma 10.3 NF1 SMARCB1
29 patent ductus arteriosus and bicuspid aortic valve with hand anomalies 10.3 MUC1 S100B
30 polyosteolysis/hyperostosis syndrome 10.3 MUC1 S100B
31 familial idiopathic steroid-resistant nephrotic syndrome with minimal changes 10.2 MUC1 S100B
32 pyoderma 10.2 CHGA MUC1
33 acute allergic serous otitis media 10.2 MUC1 S100B
34 mesenchymoma 10.2 CHGA MUC1
35 surfactant metabolism dysfunction, pulmonary, 5 10.2 NF1 SMARCB1
36 epidermolysa bullosa simplex with muscular dystrophy 10.2 MUC1 S100B
37 benallegue lacete syndrome 10.2 ENO2 S100B
38 classic variant of chromophobe renal cell carcinoma 10.2 CHGA MUC1
39 paraganglioma 10.2 CHGA NF1
40 motor neuron disease 10.2 ENO2 S100B
41 conventional malignant hemangiopericytoma 10.1 MUC1 SMARCB1
42 hydromyelia 10.1 ENO2 S100B
43 pancreatic signet ring cell adenocarcinoma 10.1 CHGA MUC1
44 mitochondrial myopathy with lactic acidosis 10.1 MUC1 NF1
45 ureter adenocarcinoma 10.1 CHGA MUC1
46 posterior urethra cancer 10.1 ENO2 SMARCB1
47 nodular medulloblastoma 10.1 ENO2 SMARCB1
48 lung benign neoplasm 10.1 ENO2 SMARCB1
49 struma ovarii 10.1 CHGA MUC1
50 mixed epithelial stromal tumour 10.1 ENO2 S100B

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 50)
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
Lenograstim Approved Phase 3 135968-09-1
5
Trabectedin Approved, Investigational Phase 2, Phase 3 114899-77-3 108150
6
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
7
Doxil Approved June 1999 Phase 3,Phase 2,Phase 1 31703
8 Alkylating Agents Phase 3,Phase 2,Phase 1
9 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
10 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
11 Isophosphamide mustard Phase 3,Phase 2,Phase 1
12 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1
13
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
14
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
15
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
16
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
17
Gemcitabine Approved Phase 2 95058-81-4 60750
18
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
19
Everolimus Approved Phase 2 159351-69-6 6442177
20
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
21
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
22
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
23
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
24
Nicotinamide Approved, Nutraceutical Phase 2 98-92-0 936
25 Micronutrients Phase 2
26 Nicotinic Acids Phase 2
27 Protein Kinase Inhibitors Phase 2,Phase 1
28 Trace Elements Phase 2
29 Vitamin B Complex Phase 2
30 Vitamins Phase 2
31 Imatinib Mesylate Phase 1, Phase 2 123596
32 Antibodies Phase 2,Phase 1
33 Antibodies, Monoclonal Phase 2,Phase 1
34 Immunoglobulins Phase 2,Phase 1
35 Anti-Infective Agents Phase 2
36 Antimetabolites Phase 2
37 Antimetabolites, Antineoplastic Phase 2
38 Antiviral Agents Phase 2
39 Immunosuppressive Agents Phase 2
40 Antimitotic Agents Phase 2
41 Protective Agents Phase 2
42 razoxane Phase 2
43 Antifungal Agents Phase 2
44 Folate Nutraceutical Phase 2
45 Vitamin B3 Nutraceutical Phase 2
46 Vitamin B9 Nutraceutical Phase 2
47 lysine Nutraceutical Phase 2,Phase 1
48
Mesna Approved 3375-50-6 598
49 Fluorodeoxyglucose F18
50 Radiopharmaceuticals

Interventional clinical trials:

(show all 26)

id Name Status NCT ID Phase Drugs
1 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Doxorubicin With or Without Ifosfamide and Pegfilgrastim in Treating Patients With Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00061984 Phase 3 doxorubicin hydrochloride;ifosfamide
3 Surgery With or Without Radiation Therapy in Treating Patients With Primary Soft Tissue Sarcoma of the Retroperitoneum or Pelvis Completed NCT00091351 Phase 3
4 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
5 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
6 Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3 doxorubicin hydrochloride;trabectedin
7 Temozolomide in Treating Patients With Advanced Soft Tissue Sarcoma Unknown status NCT00003718 Phase 2 temozolomide
8 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
9 Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma Completed NCT00245102 Phase 2 sorafenib tosylate
10 Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma Completed NCT00112463 Phase 2 romidepsin
11 STI571 in Treating Patients With Recurrent or Refractory Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
12 Ecteinascidin 743 in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00003939 Phase 2 trabectedin
13 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
14 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
15 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin;Olaratumab
16 Trial of Dasatinib in Advanced Sarcomas Active, not recruiting NCT00464620 Phase 2 Dasatinib
17 Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma Active, not recruiting NCT01614795 Phase 2 temsirolimus
18 Intensity-Modulated Radiation Therapy in Treating Patients Undergoing Surgery for Stage IB, Stage II, or Stage III Soft Tissue Sarcoma Terminated NCT00740597 Phase 2
19 Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma Completed NCT00720174 Phase 1 Doxorubicin Hydrochloride
20 Liposomal Doxorubicin Plus Ifosfamide in Treating Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00030784 Phase 1 ifosfamide;pegylated liposomal doxorubicin hydrochloride
21 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
22 Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery Recruiting NCT03009201 Phase 1 Doxorubicin;Ribociclib
23 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
24 Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas Completed NCT01567046
25 PET CT as Predictor of Response in Preoperative Chemotherapy for Soft Tissue Sarcoma Active, not recruiting NCT00346125 doxorubicin hydrochloride;ifosfamide;pegylated liposomal doxorubicin hydrochloride
26 Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma Withdrawn NCT00334984 glutamine

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:

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
A misdiagnosed melanoma: a case of cutaneous epithelioid malignant peripheral nerve sheath tumor. ( 25933073 )
2015
3
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. ( 25602794 )
2015
4
Huge epithelioid malignant peripheral nerve sheath tumor in the left axilla: a case report. ( 26366361 )
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
Advanced epithelioid malignant peripheral nerve sheath tumor showing complete response to combined surgery and chemotherapy: a case report. ( 22606446 )
2011
8
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. ( 20952276 )
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
Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). ( 9285192 )
1997
17
Purely epithelioid malignant peripheral nerve sheath tumor of the vulva. ( 9142667 )
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 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:

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
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33 ICD10
34 ICD10 via Orphanet
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65 SNOMED-CT via HPO
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