MPNST
MCID: MLG077
MIFTS: 54

Malignant Peripheral Nerve Sheath Tumor (MPNST)

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

MalaCards integrated aliases for Malignant Peripheral Nerve Sheath Tumor:

Name: Malignant Peripheral Nerve Sheath Tumor 12 20 58 6 15 17 71
Malignant Neurilemmoma 20 58 54
Neurofibrosarcoma 20 58 71
Mpnst 20 58 54
Malignant Neurofibroma 20 58
Malignant Schwannoma 20 58
Neurogenic Sarcoma 20 58
Malignant Neoplasm of the Peripheral Nerve Sheath 12
Malignant Peripheral Nerve Sheath Tumors 54

Characteristics:

Orphanet epidemiological data:

58
malignant peripheral nerve sheath tumor
Inheritance: Not applicable; Prevalence: 1-9/100000 (Worldwide); Age of onset: Adolescent,Adult,Childhood,Elderly,Infancy; Age of death: adolescent,adult,early childhood,elderly,infantile,late childhood,young Adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:5940
MeSH 44 D018319
NCIt 50 C3798
SNOMED-CT 67 134324009
ICD10 via Orphanet 33 C47.9
UMLS via Orphanet 72 C0206729 C0751690
Orphanet 58 ORPHA3148
UMLS 71 C0206729 C0751690

Summaries for Malignant Peripheral Nerve Sheath Tumor

GARD : 20 A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops in the protective lining that covers nerves. The first symptom of MPNST is often a lump or mass that increases in size, sometimes causing pain or a tingling sensation. MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung. Treatment of MPNST begins with surgery to remove as much of the tumor as possible. Radiation therapy may be used to decrease the chance of a recurrence. Chemotherapy might be used if the whole tumor cannot be removed during surgery, or to treat a metastasis. MPNSTs are quite rare, occurring in 0.001% of the general population. Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.

MalaCards based summary : Malignant Peripheral Nerve Sheath Tumor, also known as malignant neurilemmoma, is related to malignant triton tumor and epithelioid malignant peripheral nerve sheath tumor. An important gene associated with Malignant Peripheral Nerve Sheath Tumor is MXI1 (MAX Interactor 1, Dimerization Protein), and among its related pathways/superpathways are ERK Signaling and Endometrial cancer. The drugs Doxorubicin and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include skin, prostate and cervix, and related phenotypes are Decreased viability and Decreased viability

Wikipedia : 74 A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue... more...

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

Diseases in the Benign Peripheral Nerve Sheath Tumor family:

Malignant Peripheral Nerve Sheath Tumor

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

(show top 50) (show all 571)
# Related Disease Score Top Affiliating Genes
1 malignant triton tumor 33.5 S100B NF1 MYOG
2 epithelioid malignant peripheral nerve sheath tumor 33.1 SOX10 S100B NF1 MYOG CD34
3 neurofibrosarcoma 32.7 SOX10 S100A1 NF2 NF1 MXI1 CDKN2A
4 rare tumor 32.0 NF2 KIT ACTC1
5 soft tissue sarcoma 31.9 MDM2 EGFR
6 plexiform neurofibroma 31.8 S100B PDGFRA NF2 NF1 KIT CDKN2A
7 adult malignant schwannoma 31.8 NF2 NF1 MYOG CDKN2A CD34
8 neurofibroma 31.7 VIM SOX10 S100B S100A1 PDGFRA NF2
9 fibrosarcoma 31.5 VIM PDGFRA KIT ACTC1
10 neurilemmoma 31.5 VIM SOX10 S100B S100A1 PDGFRA NF2
11 perineurioma 31.4 VIM S100B S100A1 PDGFRA KIT ACTC1
12 sarcoma 31.4 VIM S100B PDGFRA NF2 MYOG MDM2
13 neurofibromatosis, type i 31.4 SOX10 S100B PDGFRA NF2 NF1 KIT
14 sarcoma, synovial 31.3 VIM S100B NF1 MYOG MDM2 KIT
15 spindle cell sarcoma 31.3 VIM S100B NF2 MYOG MDM2 KIT
16 pheochromocytoma 31.3 VIM NF1 KIT CDKN2A CDK4
17 leiomyosarcoma 31.2 VIM S100B S100A1 PDGFRA NF1 MYOG
18 spindle cell rhabdomyosarcoma 31.2 S100B S100A1 MYOG
19 neurilemmomatosis 31.2 SOX10 NF2 NF1 CDKN2A
20 melanoma 31.2 VIM SOX10 S100B PDGFRA NF2 MDM2
21 rhabdomyosarcoma 31.1 VIM S100B S100A1 PDGFRA NF1 MYOG
22 malignant fibrous histiocytoma 31.1 VIM S100B MYOG MDM2 KIT CDK4
23 cellular schwannoma 31.1 SOX10 S100A1 NF2 NF1 CD34
24 angiosarcoma 31.1 VIM MYOG KIT CD34
25 rhabdoid cancer 31.1 VIM NF2 MYOG CDKN2A ACTC1
26 connective tissue benign neoplasm 31.1 MDM2 KIT CDK4 CD34
27 mesenchymal cell neoplasm 31.0 PDGFRA MYOG KIT CD34
28 peritoneal mesothelioma 31.0 NF2 EGFR CDKN2A
29 neuroblastoma 31.0 TOP2A NF1 MDM2 KIT EGFR CDKN2A
30 benign mesothelioma 31.0 VIM NF2 CDKN2A CD34
31 chondrosarcoma 31.0 VIM S100B S100A1 PDGFRA MDM2 CDK4
32 granular cell tumor 31.0 VIM S100B S100A1
33 skin melanoma 31.0 EGFR CDKN2A CDK4 SOX10 S100B PDGFRA
34 fibrous histiocytoma 31.0 VIM S100B KIT CD34 ACTC1
35 monophasic synovial sarcoma 30.9 VIM S100B CD34
36 embryonal rhabdomyosarcoma 30.9 MYOG MDM2 CDK4
37 pilocytic astrocytoma 30.9 SOX10 S100B NF1 CDKN2A CDK4
38 sarcomatoid carcinoma 30.9 VIM MYOG KIT ACTC1
39 endometrial stromal sarcoma 30.9 VIM S100B PDGFRA KIT ACTC1
40 liposarcoma 30.9 VIM PDGFRA NF1 MYOG MDM2 CDKN2A
41 gastrointestinal stromal tumor 30.9 VIM S100B S100A1 PDGFRA NF1 KIT
42 chordoma 30.8 VIM S100B S100A1 PDGFRA EGFR CDKN2A
43 malignant spindle cell melanoma 30.8 SOX10 NF1 KIT CDKN2A CD34
44 carcinosarcoma 30.8 VIM S100A1 KIT EGFR CDKN2A ACTC1
45 plexiform schwannoma 30.8 SOX10 NF2 NF1 KIT CD34
46 glioma 30.8 PDGFRA NF1 MDM2 EGFR CDKN2A CDK4
47 pancreatic adenocarcinoma 30.8 MDM2 KIT EGFR CDKN2A CDK4
48 embryonal sarcoma 30.8 VIM S100B MYOG MDM2 KIT CDK4
49 ewing sarcoma 30.7 VIM S100B PDGFRA NF1 MYOG MDM2
50 amelanotic melanoma 30.7 VIM S100B KIT

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



Diseases related to Malignant Peripheral Nerve Sheath Tumor

Symptoms & Phenotypes for Malignant Peripheral Nerve Sheath Tumor

GenomeRNAi Phenotypes related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

26 (show all 37)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00055-A-1 9.96 EGFR
2 Decreased viability GR00055-A-2 9.96 EGFR
3 Decreased viability GR00173-A 9.96 PDGFRA
4 Decreased viability GR00221-A-1 9.96 CDKN2A EGFR KIT NF1 PDGFRA
5 Decreased viability GR00221-A-2 9.96 NF1
6 Decreased viability GR00221-A-3 9.96 CDKN2A PDGFRA
7 Decreased viability GR00221-A-4 9.96 CDKN2A EGFR NF1 PDGFRA
8 Decreased viability GR00249-S 9.96 NF1 PDGFRA
9 Decreased viability GR00301-A 9.96 KIT
10 Decreased viability GR00386-A-1 9.96 NF1
11 Decreased viability GR00402-S-2 9.96 PDGFRA
12 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.78 VIM
13 Increased shRNA abundance (Z-score > 2) GR00366-A-115 9.78 SOX10
14 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.78 EGFR
15 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.78 KIT
16 Increased shRNA abundance (Z-score > 2) GR00366-A-157 9.78 KIT
17 Increased shRNA abundance (Z-score > 2) GR00366-A-170 9.78 KIT
18 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.78 SOX10 VIM
19 Increased shRNA abundance (Z-score > 2) GR00366-A-18 9.78 SOX10
20 Increased shRNA abundance (Z-score > 2) GR00366-A-189 9.78 VIM
21 Increased shRNA abundance (Z-score > 2) GR00366-A-21 9.78 EGFR
22 Increased shRNA abundance (Z-score > 2) GR00366-A-213 9.78 S100B
23 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.78 EGFR
24 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.78 KIT
25 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.78 EGFR
26 Increased shRNA abundance (Z-score > 2) GR00366-A-48 9.78 S100B
27 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.78 SOX10
28 Increased shRNA abundance (Z-score > 2) GR00366-A-61 9.78 KIT
29 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.78 VIM
30 Increased shRNA abundance (Z-score > 2) GR00366-A-66 9.78 S100B
31 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.78 SOX10
32 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.78 CDK4 EGFR KIT SOX10
33 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.78 KIT
34 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.78 EGFR SOX10
35 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.78 KIT
36 Decreased cell migration GR00055-A-1 9.46 CDK4 NF2 VIM
37 Decreased cell migration GR00055-A-3 9.46 EGFR

MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

46 (show all 26)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.52 ACTC1 CD34 CDK4 CDKN2A EGFR KIT
2 cellular MP:0005384 10.46 ACTC1 CD34 CDK4 CDKN2A EGFR KIT
3 behavior/neurological MP:0005386 10.44 CDK4 CDKN2A KIT MDM2 MYOG NF1
4 embryo MP:0005380 10.44 ACTC1 CDK4 CDKN2A EGFR KIT MDM2
5 growth/size/body region MP:0005378 10.43 ACTC1 CDK4 CDKN2A EGFR HTRA1 KIT
6 cardiovascular system MP:0005385 10.4 ACTC1 CDK4 CDKN2A EGFR HTRA1 KIT
7 hematopoietic system MP:0005397 10.39 ACTC1 CD34 CDK4 CDKN2A EGFR KIT
8 mortality/aging MP:0010768 10.39 ACTC1 CDK4 CDKN2A EGFR KIT MDM2
9 immune system MP:0005387 10.37 CD34 CDK4 CDKN2A EGFR KIT MDM2
10 integument MP:0010771 10.36 CD34 CDK4 CDKN2A EGFR KIT MDM2
11 endocrine/exocrine gland MP:0005379 10.33 CDK4 CDKN2A EGFR KIT MDM2 MXI1
12 muscle MP:0005369 10.27 ACTC1 CDK4 CDKN2A EGFR KIT MDM2
13 craniofacial MP:0005382 10.26 ACTC1 CDK4 EGFR KIT MDM2 NF1
14 digestive/alimentary MP:0005381 10.26 CDK4 CDKN2A EGFR KIT MDM2 NF1
15 nervous system MP:0003631 10.23 ACTC1 CDK4 CDKN2A EGFR KIT MDM2
16 neoplasm MP:0002006 10.22 CD34 CDK4 CDKN2A EGFR KIT MDM2
17 normal MP:0002873 10.18 ACTC1 CDK4 EGFR KIT MDM2 MYOG
18 limbs/digits/tail MP:0005371 10.14 EGFR KIT MDM2 NF1 PDGFRA SH3PXD2A
19 liver/biliary system MP:0005370 10.13 CDK4 CDKN2A EGFR KIT MDM2 NF1
20 reproductive system MP:0005389 10 CDK4 CDKN2A EGFR KIT MDM2 MXI1
21 no phenotypic analysis MP:0003012 9.98 CDKN2A EGFR KIT MDM2 MYOG PDGFRA
22 pigmentation MP:0001186 9.97 CDK4 CDKN2A EGFR KIT MDM2 NF1
23 renal/urinary system MP:0005367 9.97 CDK4 EGFR KIT MDM2 MXI1 NF1
24 respiratory system MP:0005388 9.9 CDKN2A EGFR KIT MYOG NF1 NF2
25 skeleton MP:0005390 9.73 CDK4 CDKN2A EGFR HTRA1 KIT MDM2
26 vision/eye MP:0005391 9.32 CDK4 CDKN2A EGFR HTRA1 KIT NF1

Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

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

(show top 50) (show all 81)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
2
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
3
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
6 Antibiotics, Antitubercular Phase 2, Phase 3
7 Anti-Bacterial Agents Phase 2, Phase 3
8
Liposomal doxorubicin Phase 2, Phase 3 31703
9
Isophosphamide mustard Phase 2, Phase 3 100427
10 Alkylating Agents Phase 2, Phase 3
11 Protein Kinase Inhibitors Phase 2, Phase 3
12 Imatinib Mesylate Phase 2, Phase 3 220127-57-1 123596
13
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
14
Bevacizumab Approved, Investigational Phase 2 216974-75-3
15
Xylometazoline Approved, Investigational Phase 1, Phase 2 526-36-3 5709
16
Lenograstim Approved, Investigational Phase 2 135968-09-1
17
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
18
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
19
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
20
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166 2942
21
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
22
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
23
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
24
Sargramostim Approved, Investigational Phase 1, Phase 2 123774-72-1, 83869-56-1
25
Pembrolizumab Approved Phase 2 1374853-91-4
26
Ipilimumab Approved Phase 2 477202-00-9
27
nivolumab Approved Phase 2 946414-94-4
28
Etoposide Approved Phase 2 33419-42-0 36462
29
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 445643 439492 6473866
30
Melphalan Approved Phase 2 148-82-3 4053 460612
31
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
32
Mycophenolic acid Approved Phase 2 24280-93-1 446541
33
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
34
Adenosine Approved, Investigational Phase 2 58-61-7 60961
35
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
36
Mesna Approved, Investigational Phase 2 3375-50-6 598
37
Gemcitabine Approved Phase 1, Phase 2 95058-81-4 60750
38
Docetaxel Approved, Investigational Phase 1, Phase 2 114977-28-5 148124
39
Threonine Approved, Nutraceutical Phase 2 72-19-5 6288
40
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
41
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
42 Lorvotuzumab mertansine Investigational Phase 2 1008106-64-6
43 Angiogenesis Inhibitors Phase 2
44
Erlotinib Hydrochloride Phase 2 183319-69-9 176871
45 Antineoplastic Agents, Immunological Phase 2
46 Antifungal Agents Phase 2
47 Mitogens Phase 2
48 Etoposide phosphate Phase 2
49 Protective Agents Phase 2
50 Cardiotonic Agents Phase 2

Interventional clinical trials:

(show all 47)
# Name Status NCT ID Phase Drugs
1 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Unknown status NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NSC# 737754) Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
3 An Open-label Multicenter Phase II Study of Imatinib Mesylate Treatment of Patients With Malignant Peripheral Nerve Sheath Tumors Terminated NCT00427583 Phase 2, Phase 3 imatinib mesylate
4 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
5 Phase 2 Study of the mTOR Inhibitor Everolimus in Combination With Bevacizumab in Patients With Sporadic and Neurofibromatosis Type 1 (NF1) Related Refractory Malignant Peripheral Nerve Sheath Tumors Completed NCT01661283 Phase 2 everolimus;bevacizumab
6 Phase I/II Trial of Torisel and Liposomal Doxorubicin in Patients With Advanced Soft Tissue and Bone Sarcomas Completed NCT00949325 Phase 1, Phase 2 temsirolimus plus liposomal doxorubicin
7 A Phase I/II Trial of Ganetespib in Combination With the mTOR Inhibitor Sirolimus for Patients With Recurrent or Refractory Sarcomas Including Unresectable or Metastatic Malignant Peripheral Nerve Sheath Tumors Completed NCT02008877 Phase 1, Phase 2 ganetespib;Sirolimus
8 Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated High Grade Malignant Peripheral Nerve Sheath Tumors Completed NCT00304083 Phase 2 doxorubicin hydrochloride;etoposide;ifosfamide
9 U.S./Canada Sarcoma Intergroup Study of OSI-774 in Malignant Peripheral Nerve Sheath Tumors, Phase II Completed NCT00068367 Phase 2 erlotinib hydrochloride
10 A Phase 1B/II Study of GDC-0449 (NSC 747691) in Combination With RO4929097, a Gamma-Secretase Inhibitor (GSI) in Advanced/Metastatic Sarcomas Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
11 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
12 A Phase II Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
13 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
14 A Randomized, Double-Blind Phase II, Study of Gemcitabine Alone or in Combination With Pazopanib for Refractory Soft Tissue Sarcoma Completed NCT01532687 Phase 2 Gemcitabine;Gemcitabine Hydrochloride;Pazopanib;Pazopanib Hydrochloride
15 A Phase II Study of Cixutumumab (IMC-A12) in Combination With Temsirolimus in Pediatric Patients With Recurrent or Refractory Solid Tumors Completed NCT01614795 Phase 2 Temsirolimus
16 Phase II Trial of Sorafenib and Dacarbazine in Soft Tissue Sarcoma Completed NCT00837148 Phase 2 Sorafenib and Dacarbazine
17 Phase II Study of MLN8237 in Advanced/Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
18 A Phase II Study of Pembrolizumab in Patients With Malignant Peripheral Nerve Sheath Tumor (MPNST), Not Eligible for Curative Surgery Recruiting NCT02691026 Phase 2 Pembrolizumab
19 A Phase II Basket Trial of Glutaminase Inhibitor (BeGIN) CB-839 HCl in Patients With NF1 Aberrations, NF1 Mutant Malignant Peripheral Nerve Sheath Tumors (MPNST), KEAP1/NRF2 and LKB1 Aberrant Tumors Recruiting NCT03872427 Phase 2 Telaglenastat Hydrochloride
20 SARC031: A Phase 2 Trial of the MEK Inhibitor Selumetinib (AZD6244 Hydrogen Sulfate) in Combination With the mTOR Inhibitor Sirolimus for Patients With Unresectable or Metastatic Malignant Peripheral Nerve Sheath Tumors Recruiting NCT03433183 Phase 2 Selumetinib;Sirolimus
21 Phase I Study Evaluating Combination Therapy With the Receptor Tyrosine Kinase Inhibitor PLX3397 and Sirolimus in Patients With Unresectable Sarcoma and Phase II Study in Malignant Peripheral Nerve Sheath Tumors Recruiting NCT02584647 Phase 1, Phase 2 PLX3397;Sirolimus
22 A Non-Inferiority Study of Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin
23 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
24 A Pilot Study of Allogeneic Hematopoietic Stem Cell Transplantation for Pediatric and Adolescent-Young Adults Patients With High Risk Solid Tumors Recruiting NCT04530487 Phase 2 Cyclosporine;Etoposide;Fludarabine Phosphate;Melphalan;Mycophenolate Mofetil;Tacrolimus;Thiotepa
25 A Phase 2 Study of IMGN901 (Lorvotuzumab Mertansine; NSC#: 783609) in Children With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor (MPNST) and Synovial Sarcoma Active, not recruiting NCT02452554 Phase 2
26 A Phase I/Randomized Phase II Study of MLN0128 (TAK-228) VS. Pazopanib in Patients With Locally Advanced/Unresectable and/or Metastatic Sarcoma Active, not recruiting NCT02601209 Phase 1, Phase 2 Pazopanib;Pazopanib Hydrochloride;Sapanisertib
27 Hypofractionated Radiotherapy With Hyperthermia in Unresectable or Marginally Resectable Soft Tissue Sarcomas Active, not recruiting NCT03989596 Phase 2
28 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Active, not recruiting NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
29 A Phase IB/II Study of Gemcitabine and Docetaxel in Combination With Pazopanib (Gem/Doce/Pzb) for the Neoadjuvant Treatment of Soft Tissue Sarcoma (STS) Terminated NCT01418001 Phase 1, Phase 2 Pazopanib 400 mg QD - Gemcitabine and Docetaxel in Combination
30 A Phase 2 Study of CPI-0610, a Small Molecule Inhibitor of Bromodomain and Extra-Terminal (BET) Proteins, in Patients With Malignant Peripheral Nerve Sheath Tumors Withdrawn NCT02986919 Phase 2 CPI-0610
31 A Phase I, Open-Label, Dose Escalation Study of JX-594 (Vaccinia GM-CSF/Thymidine Kinase-Deactivated Virus) Administered by Intratumoral Injection in Pediatric Patients With Unresectable Refractory Solid Tumors. Completed NCT01169584 Phase 1 Recombinant Vaccinia GM-CSF; RAC VAC GM-CSF (JX-594)
32 Transformation of Plexiform Neurofibromas to Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1: Clinical, Histopathologic, and Genomic Analysis Completed NCT02211768 Phase 1 [18F]-FLT-PET/CT scans
33 A Phase 1 Study of Doxorubicin and A12 in Advanced Soft Tissue Sarcoma Completed NCT00720174 Phase 1 Doxorubicin Hydrochloride
34 A Phase I Dose Escalation Study of Intratumoral or Intravenous Herpes Simplex Virus-1 Mutant HSV1716 in Patients With Refractory Non-Central Nervous System (Non-CNS) Solid Tumors Completed NCT00931931 Phase 1
35 Phase I Trial of Intratumoral Administration of a NIS-Expressing Derivative Manufactured From a Genetically Engineered Strain of Measles Virus in Patients With Unresectable or Recurrent Malignant Peripheral Nerve Sheath Tumor Recruiting NCT02700230 Phase 1
36 Phase I Study of EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT03618381 Phase 1
37 First-in-Human Phase I Trial to Investigate the Safety, Tolerability, Pharmacokinetics, Biological and Clinical Activity of Metarrestin (ML-246) in Subjects With Metastatic Solid Tumors. Recruiting NCT04222413 Phase 1 Metarrestin
38 Phase I Study of B7H3 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT04483778 Phase 1
39 A Phase 1B Study of Ribociclib in Combination With Doxorubicin in Advanced Soft Tissue Sarcomas Active, not recruiting NCT03009201 Phase 1 Doxorubicin Hydrochloride;Ribociclib
40 Neoadjuvant Nivolumab Plus Ipilimumab for Newly Diagnosed Malignant Peripheral Nerve Sheath Tumor Not yet recruiting NCT04465643 Phase 1 Nivolumab;Ipilimumab
41 A Phase I Study of the Selective Inhibitor of Nuclear Export (SINE) Selinexor in Combination With the Proteasome Inhibitor Ixazomib for the Treatment of Advanced Sarcoma Withdrawn NCT03880123 Phase 1 Selinexor;Ixazomib
42 Analysis of Circulating Tumor DNA in Plasma of Neurofibromatosis Type 1 Patients With MPNSTs Using Microarray CGH Unknown status NCT01218152
43 Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis Unknown status NCT01777451
44 Observational - Potentially Actionable Mutations in Archived Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) Completed NCT01567046
45 Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors Recruiting NCT03141021
46 Natural History Study and Longitudinal Assessment of Children, Adolescents, and Adults With Neurofibromatosis Type 1 Active, not recruiting NCT00924196
47 BLESSED: Expanded Access for DeltaRex-G for Advanced Pancreatic Cancer and Sarcoma Available NCT04091295 DeltaRex-G

Search NIH Clinical Center for Malignant Peripheral Nerve Sheath Tumor

Genetic Tests for Malignant Peripheral Nerve Sheath Tumor

Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

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

40
Skin, Prostate, Cervix, Pancreas, Bone, Spinal Cord, Colon

Publications for Malignant Peripheral Nerve Sheath Tumor

Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50) (show all 1645)
# Title Authors PMID Year
1
Mxi1 mutations in human neurofibrosarcomas. 6 61
10470286 1999
2
Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. 54 61
20399579 2010
3
Tyrosine kinase expression in pulmonary metastases and paired primary tumors. 61 54
19948538 2010
4
The neurofibromatosis type 1 tumor suppressor controls cell growth by regulating signal transducer and activator of transcription-3 activity in vitro and in vivo. 61 54
20124472 2010
5
Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft. 61 54
19634141 2010
6
Establishment and characterization of a novel human malignant peripheral nerve sheath tumor cell line, FMS-1, that overexpresses epidermal growth factor receptor and cyclooxygenase-2. 54 61
19921253 2009
7
Malignant peripheral nerve sheath tumor of the uterine cervix expressing both S-100 protein and HMB-45. 61 54
20025639 2009
8
Neuregulin-1 beta and neuregulin-1 alpha differentially affect the migration and invasion of malignant peripheral nerve sheath tumor cells. 61 54
19306381 2009
9
Expression and significance of EGFR in malignant peripheral nerve sheath tumor. 61 54
19330289 2009
10
High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization. 54 61
18281533 2008
11
Malignant peripheral nerve sheath tumor of the breast: case report. 54 61
18154670 2007
12
Primary pulmonary malignant peripheral nerve sheath tumor: a case report. 54 61
17910355 2007
13
Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB. 54 61
17558420 2007
14
Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation. 54 61
17119986 2007
15
Nestin expression as a new marker in malignant peripheral nerve sheath tumors. 54 61
17300669 2007
16
Oncolytic HSV and erlotinib inhibit tumor growth and angiogenesis in a novel malignant peripheral nerve sheath tumor xenograft model. 61 54
17235305 2007
17
Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. 54 61
16528378 2006
18
Calretinin expression in tumors of adipose tissue. 54 61
16613326 2006
19
Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. 61 54
16510576 2006
20
Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures. 54 61
16481232 2006
21
ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas. 54 61
15609299 2005
22
[Morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors]. 61 54
15774214 2005
23
CD44-independent hepatocyte growth factor/c-Met autocrine loop promotes malignant peripheral nerve sheath tumor cell invasion in vitro. 61 54
14730703 2004
24
Topoisomerase-II alpha is upregulated in malignant peripheral nerve sheath tumors and associated with clinical outcome. 61 54
14673046 2003
25
Protein gene product 9.5 (PGP 9.5) is not a specific marker of neural and nerve sheath tumors: an immunohistochemical study of 95 mesenchymal neoplasms. 54 61
14559978 2003
26
Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions. 54 61
14508395 2003
27
p15INK4b, p14ARF, and p16INK4a inactivation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. 54 61
14519636 2003
28
Constitutive activation of the neuregulin-1/ErbB receptor signaling pathway is essential for the proliferation of a neoplastic Schwann cell line. 54 61
12838503 2003
29
Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. 61 54
12730955 2003
30
Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors. 54 61
12692193 2003
31
Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms. 54 61
12152785 2002
32
Immunohistochemical staining for KIT (CD117) in soft tissue sarcomas is very limited in distribution. 61 54
11865845 2002
33
Are myogenin and myoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics. 61 54
11688574 2001
34
Malignant melanoma with a myxoid stroma: a diagnostic pitfall on fine-needle aspiration biopsy. 54 61
11536443 2001
35
Soft tissue sarcomas in the African hedgehog (Atelerix albiventris): microscopic and immunohistologic study of three cases. 54 61
11580072 2001
36
NF1 deletions in S-100 protein-positive and negative cells of sporadic and neurofibromatosis 1 (NF1)-associated plexiform neurofibromas and malignant peripheral nerve sheath tumors. 61 54
11438454 2001
37
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. 61 54
11145248 2001
38
Microphthalmia transcription factor and melanoma cell adhesion molecule expression distinguish desmoplastic/spindle cell melanoma from morphologic mimics. 54 61
11145252 2001
39
Epidermal growth factor receptor expression in neurofibromatosis type 1-related tumors and NF1 animal models. 61 54
10791998 2000
40
Metastatic malignant melanoma resembling malignant peripheral nerve sheath tumor: report of 16 cases. 54 61
10584703 1999
41
Usefulness of cytokeratin subsets for distinguishing monophasic synovial sarcoma from malignant peripheral nerve sheath tumor. 54 61
10549251 1999
42
Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. 54 61
10469433 1999
43
CD44 expression is aberrant in benign Schwann cell tumors possessing mutations in the neurofibromatosis type 2, but not type 1, gene. 54 61
9354454 1997
44
The Interval Between Preoperative Radiation and Surgery Is Not Associated with Overall Survival for Soft-tissue Sarcomas: An Analysis of the National Cancer Database. 61
32401002 2021
45
LMNA-NTRK1 rearranged mesenchymal tumor (lipofibromatosis-like neural tumor) mimicking pigmented dermatofibrosarcoma protuberans. 61
32519338 2021
46
Cytomorphologic Spectrum of SMARCB1-Deficient Soft Tissue Neoplasms. 61
33608696 2021
47
Temporal regulation of tumor growth in nocturnal mammals: In vivo studies and chemotherapeutical potential. 61
33428275 2021
48
Intracranial metastasis from a malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1: A case study and literature review. 61
33607580 2021
49
Chromosome 8 gain is associated with high-grade transformation in MPNST. 61
33591953 2021
50
Repetitive syncope caused by a rare massive sporadic malignant peripheral nerve sheath tumor involving carotid arteries: A case report. 61
33592886 2021

Variations for Malignant Peripheral Nerve Sheath Tumor

ClinVar genetic disease variations for Malignant Peripheral Nerve Sheath Tumor:

6
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 MXI1 NM_130439.3(MXI1):c.362C>T (p.Ala121Val) SNV Pathogenic 9537 rs137852604 10:111988034-111988034 10:110228276-110228276

Copy number variations for Malignant Peripheral Nerve Sheath Tumor from CNVD:

7 (show all 45)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 17387 1 142600000 147000000 Gain Malignant peripheral nerve sheath tumor
2 22132 1 165500000 170900000 Gain Malignant peripheral nerve sheath tumor
3 23047 1 170900000 176000000 Gain Malignant peripheral nerve sheath tumor
4 49109 11 102900000 112500000 Loss Malignant peripheral nerve sheath tumor
5 49995 11 112500000 121200000 Loss Malignant peripheral nerve sheath tumor
6 53584 11 31000000 36400000 Loss Malignant peripheral nerve sheath tumor
7 61507 12 1 3100000 Gain FOXM1 Malignant peripheral nerve sheath tumor
8 65742 12 21200000 26300000 Amplification SOX5 Malignant peripheral nerve sheath tumor
9 66816 12 3300000 10100000 Gain Malignant peripheral nerve sheath tumor
10 67235 12 38200000 49100000 Gain Malignant peripheral nerve sheath tumor
11 69080 12 5300000 10000000 Gain NOP2 Malignant peripheral nerve sheath tumor
12 69882 12 56428269 56432431 Gain CDK4 Malignant peripheral nerve sheath tumor
13 69960 12 56600000 71500000 Gain Malignant peripheral nerve sheath tumor
14 71535 12 71500000 86700000 Gain Malignant peripheral nerve sheath tumor
15 73254 12 92600000 109000000 Gain Malignant peripheral nerve sheath tumor
16 73259 12 92600000 96200000 Gain Malignant peripheral nerve sheath tumor
17 75380 13 19500000 25500000 Gain Malignant peripheral nerve sheath tumor
18 76637 13 34000000 55300000 Gain Malignant peripheral nerve sheath tumor
19 79536 13 73300000 77200000 Gain Malignant peripheral nerve sheath tumor
20 85488 14 47200000 67900000 Loss Malignant peripheral nerve sheath tumor
21 87451 14 73800000 107349540 Gain Malignant peripheral nerve sheath tumor
22 97234 16 1 10500000 Gain Malignant peripheral nerve sheath tumor
23 102841 16 57400000 90354753 Gain Malignant peripheral nerve sheath tumor
24 105377 16 7900000 14800000 Gain Malignant peripheral nerve sheath tumor
25 115613 17 58300000 81195210 Gain Malignant peripheral nerve sheath tumor
26 124065 19 1 13900000 Gain Malignant peripheral nerve sheath tumor
27 147242 2 61300000 68600000 Gain Malignant peripheral nerve sheath tumor
28 150135 2 96800000 114400000 Gain Malignant peripheral nerve sheath tumor
29 163892 22 32200000 44200000 Gain Malignant peripheral nerve sheath tumor
30 172609 3 184500000 198022430 Gain Malignant peripheral nerve sheath tumor
31 174546 3 2800000 16400000 Gain Malignant peripheral nerve sheath tumor
32 175421 3 39400000 54400000 Gain Malignant peripheral nerve sheath tumor
33 196162 5 159900000 180915260 Gain Malignant peripheral nerve sheath tumor
34 198008 5 18400000 23300000 Gain Malignant peripheral nerve sheath tumor
35 209673 6 27000000 57000000 Gain Malignant peripheral nerve sheath tumor
36 213890 6 52900000 70000000 Gain Malignant peripheral nerve sheath tumor
37 220382 7 13800000 43300000 Gain Malignant peripheral nerve sheath tumor
38 227906 7 72200000 86400000 Gain Malignant peripheral nerve sheath tumor
39 240607 8 52600000 146364022 Gain Malignant peripheral nerve sheath tumor
40 241476 8 6200000 36500000 Gain Malignant peripheral nerve sheath tumor
41 244863 9 1 14200000 Gain Malignant peripheral nerve sheath tumor
42 247559 9 130300000 135900000 Gain Malignant peripheral nerve sheath tumor
43 249423 9 14200000 28000000 Loss Malignant peripheral nerve sheath tumor
44 255080 9 72339785 73251640 Dramatically overexpressed,genomicallyamplified TRPM3 Malignant peripheral nerve sheath tumor
45 255774 9 84100000 102600000 Gain Malignant peripheral nerve sheath tumor

Expression for Malignant Peripheral Nerve Sheath Tumor

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

Pathways for Malignant Peripheral Nerve Sheath Tumor

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

(show all 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.69 VIM SOX10 PDGFRA NRG1 NF1 MDM2
2
Show member pathways
12.7 PDGFRA MDM2 KIT EGFR CDKN2A CDK4
3
Show member pathways
12.62 PDGFRA NRG1 MDM2 KIT EGFR
4 12.61 PDGFRA MDM2 KIT EGFR CDKN2A CDK4
5
Show member pathways
12.51 PDGFRA NRG1 NF1 MDM2 EGFR
6 12.4 VIM PDGFRA MDM2 EGFR CDKN2A
7
Show member pathways
12.4 PDGFRA NF1 MDM2 EGFR CDKN2A CDK4
8 12.33 VIM TOP2A MDM2 CDKN2A CDK4
9 12.04 NF1 MDM2 EGFR CDK4
10 12.01 PDGFRA NRG1 KIT EGFR
11
Show member pathways
11.91 NRG1 NF1 EGFR CDK4
12 11.73 TOP2A MDM2 CDK4
13
Show member pathways
11.7 S100B NRG1 MDM2 EGFR
14 11.64 TOP2A MDM2 CDKN2A
15 11.61 PDGFRA KIT EGFR
16 11.49 PDGFRA NF1 CDK4
17 11.46 VIM SOX10 S100B PDGFRA
18 11.45 PDGFRA EGFR CDKN2A
19 11.32 MDM2 EGFR CDKN2A CDK4
20 11.21 PDGFRA NF2 NF1 MDM2 EGFR CDKN2A
21 11.11 MDM2 CDKN2A CDK4
22 10.98 PDGFRA NRG1 KIT ACTC1

GO Terms for Malignant Peripheral Nerve Sheath Tumor

Cellular components related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 10.03 VIM TOP2A SOX10 S100B S100A1 PDGFRA
2 nucleoplasm GO:0005654 10 TOP2A SOX10 S100B S100A1 PDGFRA NRG1
3 nucleolus GO:0005730 9.87 TOP2A NF2 NF1 MXI1 MDM2 CDKN2A
4 cytoplasm GO:0005737 9.58 VIM TOP2A SOX10 SH3PXD2A S100B S100A1
5 protein-containing complex GO:0032991 9.5 TOP2A S100A1 PDGFRA MDM2 EGFR CDKN2A

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

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of protein kinase activity GO:0006469 9.72 NF2 NF1 CDKN2A
2 MAPK cascade GO:0000165 9.72 PDGFRA NRG1 NF1 KIT EGFR
3 positive regulation of protein kinase B signaling GO:0051897 9.71 PDGFRA NRG1 KIT EGFR
4 positive regulation of kinase activity GO:0033674 9.67 PDGFRA KIT EGFR
5 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.67 PDGFRA NRG1 KIT EGFR
6 cellular response to growth factor stimulus GO:0071363 9.63 MYOG MDM2 EGFR
7 hematopoietic progenitor cell differentiation GO:0002244 9.61 TOP2A PDGFRA KIT
8 positive regulation of phospholipase C activity GO:0010863 9.56 PDGFRA KIT
9 amyloid fibril formation GO:1990000 9.54 MDM2 CDKN2A
10 cardiac myofibril assembly GO:0055003 9.52 PDGFRA ACTC1
11 tongue development GO:0043586 9.51 KIT EGFR
12 positive regulation of cell proliferation GO:0008284 9.5 S100B PDGFRA NRG1 MDM2 KIT EGFR
13 somatic stem cell division GO:0048103 9.48 KIT CDKN2A
14 wound healing GO:0042060 9.46 PDGFRA NRG1 NF1 EGFR
15 peripheral nervous system development GO:0007422 9.43 SOX10 NRG1 NF1
16 positive regulation of gene expression GO:0010628 9.23 VIM SOX10 NRG1 MDM2 KIT CDKN2A

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.93 VIM TOP2A SOX10 SH3PXD2A S100B S100A1
2 transmembrane receptor protein tyrosine kinase activity GO:0004714 8.8 PDGFRA KIT EGFR

Sources for Malignant Peripheral Nerve Sheath Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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