MCID: NRF003
MIFTS: 45

Neurofibrosarcoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Neurofibrosarcoma

MalaCards integrated aliases for Neurofibrosarcoma:

Name: Neurofibrosarcoma 11 19 75 53 5 43 14 71 75
Malignant Peripheral Nerve Sheath Tumor 71
Neurosarcoma 11

Classifications:



External Ids:

Disease Ontology 11 DOID:3512
MeSH 43 D018319
SNOMED-CT 68 19897006
UMLS 71 C0206729 C0751690

Summaries for Neurofibrosarcoma

MalaCards based summary: Neurofibrosarcoma, also known as malignant peripheral nerve sheath tumor, is related to childhood malignant schwannoma and malignant peripheral nerve sheath tumor. An important gene associated with Neurofibrosarcoma is MXI1 (MAX Interactor 1, Dimerization Protein), and among its related pathways/superpathways are Endometrial cancer and Malignant pleural mesothelioma. The drugs Ifosfamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include t cells, small intestine and breast, and related phenotypes are Decreased viability and Increased proliferation

Wikipedia: 75 A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue... more...

Related Diseases for Neurofibrosarcoma

Diseases related to Neurofibrosarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 425)
# Related Disease Score Top Affiliating Genes
1 childhood malignant schwannoma 31.7 SUZ12 NF1
2 malignant peripheral nerve sheath tumor 30.7 TP53 SOX10 NF2 NF1 KIT CDKN2A
3 meckel diverticulum 29.9 KIT CD34
4 pleomorphic adenoma 29.8 TP53 FGFR1 ENO2
5 adult malignant schwannoma 29.8 TP53 SUZ12 SOX10 NF1 KIT CDKN2A
6 mesenchymal cell neoplasm 29.8 TP53 NF1 KIT CD34
7 neurofibromatosis 29.8 TP53 SUZ12 NF2 NF1 KIT CDKN2A
8 sarcoma, synovial 29.7 NF1 KIT CD34
9 sarcoma 29.7 TP53 SUZ12 KIT FGFR1 ENO2
10 neurilemmoma 29.6 SOX10 NF2 NF1 KIT ENO2 CD34
11 cutaneous fibrous histiocytoma 29.6 SOX10 ENO2 CD34
12 lipomatosis, multiple 29.5 TP53 NF1 KIT CD34
13 malignant triton tumor 29.5 SOX10 NF1 ENO2 CD34
14 thymoma 29.3 TP53 KIT CDKN2A
15 neurofibroma 29.3 TP53 SOX10 NF2 NF1 KIT CDKN2A
16 endometrial stromal sarcoma 29.2 TP53 SUZ12 KIT CD34
17 leiomyosarcoma 29.2 TP53 NF1 KIT ENO2 CDKN2A
18 neuroma 29.1 NF2 NF1 KIT FGFR1 ENO2 CD34
19 mixed cell type cancer 29.0 TP53 KIT ENO2 CDKN2A
20 hemangioma 28.7 TP53 KIT ENO2 CDKN2A CD34
21 rhabdomyosarcoma 28.6 TP53 NF1 KIT FGFR1 ENO2 CDKN2A
22 neurofibromatosis, type i 28.5 TP53 SUZ12 NFIC NF2 NF1 KIT
23 wilms tumor 1 28.5 TP53 NF1 KIT ENO2 CDKN2A CD34
24 plexiform neurofibroma 27.9 TP53 SUZ12 SOX10 NFIC NF2 NF1
25 gastrointestinal stromal tumor 27.7 TP53 SOX10 NF2 NF1 KIT FGFR1
26 meningioma, familial 27.6 TP73 TP53 SOX10 NF2 NF1 KIT
27 neurofibromatosis-noonan syndrome 10.6
28 pilocytic astrocytoma of cerebellum 10.3 TACC1 NF1
29 peripheral nerve schwannoma 10.3 NF2 NF1
30 optic nerve sheath meningioma 10.3 NF2 NF1
31 spinal cord astrocytoma 10.3 NF2 NF1
32 polyp of corpus uteri 10.3 NF2 NF1
33 cerebellar astrocytoma 10.3 TACC1 NF1
34 childhood pilocytic astrocytoma 10.3 NF1 FGFR1
35 schwannoma of twelfth cranial nerve 10.3 NF2 NF1
36 hypoglossal nerve disease 10.3 NF2 NF1
37 rete ovarii adenoma 10.3 NF2 NF1
38 rete ovarii benign neoplasm 10.3 NF2 NF1
39 subungual glomus tumor 10.3 NF1 CD34
40 spinal canal and spinal cord meningioma 10.3 NF2 NF1
41 central nervous system fibrosarcoma 10.3 NF2 CD34
42 spinal meningioma 10.3 NF2 NF1
43 lung meningioma 10.3 NF2 CD34
44 psammomatous meningioma 10.3 NF2 CD34
45 meninges hemangiopericytoma 10.3 NF2 CD34
46 angiomatous meningioma 10.3 NF2 CD34
47 chordoid meningioma 10.2 NF2 CD34
48 juvenile pilocytic astrocytoma 10.2 TP53 NF1
49 scrotum neoplasm 10.2 NFIC CD34
50 malignant dermis tumor 10.2 NF1 KIT

Graphical network of the top 20 diseases related to Neurofibrosarcoma:



Diseases related to Neurofibrosarcoma

Symptoms & Phenotypes for Neurofibrosarcoma

GenomeRNAi Phenotypes related to Neurofibrosarcoma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00107-A-1 9.68 DCK
2 Decreased viability GR00221-A-1 9.68 CDKN2A DCK FGFR1 KIT NF1 SUZ12
3 Decreased viability GR00221-A-2 9.68 DCK FGFR1 NF1 SUZ12
4 Decreased viability GR00221-A-3 9.68 CDKN2A
5 Decreased viability GR00221-A-4 9.68 CDKN2A DCK NF1
6 Decreased viability GR00240-S-1 9.68 DCK
7 Decreased viability GR00249-S 9.68 NF1 SUZ12
8 Decreased viability GR00301-A 9.68 KIT
9 Decreased viability GR00386-A-1 9.68 DCK FGFR1 NF1 SUZ12
10 Increased proliferation GR00094-A 8.96 NF2 TP53

MGI Mouse Phenotypes related to Neurofibrosarcoma:

45 (show all 17)
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 10.29 CDKN2A ENO2 FGFR1 KIT NF1 NF2
2 neoplasm MP:0002006 10.26 CD34 CDKN2A KIT MXI1 NF1 NF2
3 growth/size/body region MP:0005378 10.26 CDKN2A ENO2 FGFR1 KIT NF1 NF2
4 endocrine/exocrine gland MP:0005379 10.21 CDKN2A DCK FGFR1 KIT MXI1 NF1
5 cellular MP:0005384 10.21 CD34 CDKN2A DCK ENO2 FGFR1 KIT
6 behavior/neurological MP:0005386 10.18 CDKN2A ENO2 FGFR1 KIT NF1 NF2
7 embryo MP:0005380 10.15 CDKN2A FGFR1 KIT NF1 NF2 SOX10
8 pigmentation MP:0001186 10.11 CDKN2A KIT NF1 NF2 SOX10 SUZ12
9 immune system MP:0005387 10.11 CD34 CDKN2A DCK FGFR1 KIT MXI1
10 digestive/alimentary MP:0005381 10.08 CDKN2A FGFR1 KIT NF1 NF2 SOX10
11 craniofacial MP:0005382 10.06 ENO2 FGFR1 KIT NF1 NF2 NFIC
12 hearing/vestibular/ear MP:0005377 10.01 FGFR1 KIT NF1 NF2 TP53 TP73
13 respiratory system MP:0005388 9.92 CDKN2A ENO2 KIT NF1 NF2 SOX10
14 reproductive system MP:0005389 9.91 CDKN2A FGFR1 KIT MXI1 NF1 NF2
15 skeleton MP:0005390 9.81 CDKN2A FGFR1 KIT NF1 NF2 NFIC
16 hematopoietic system MP:0005397 9.65 CD34 CDKN2A DCK FGFR1 KIT MXI1
17 integument MP:0010771 9.23 CD34 CDKN2A FGFR1 KIT NF1 NF2

Drugs & Therapeutics for Neurofibrosarcoma

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

(show top 50) (show all 89)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
6
Palifosfamide Investigational Phase 2, Phase 3 31645-39-3 100427
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Alkylating Agents Phase 2, Phase 3
9 Antineoplastic Agents, Alkylating Phase 2, Phase 3
10 Antibiotics, Antitubercular Phase 2, Phase 3
11 Liposomal doxorubicin Phase 2, Phase 3
12 Protein Kinase Inhibitors Phase 2, Phase 3
13
Imatinib Mesylate Phase 2, Phase 3 220127-57-1
14
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354
15
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239
16
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
17
Xylometazoline Approved, Investigational Phase 1, Phase 2 526-36-3 5709
18
Dacarbazine Approved, Investigational Phase 2 4342-03-4 2942 5351166
19
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
20
Adenosine Approved, Investigational Phase 2 58-61-7 60961
21
Trastuzumab Approved, Investigational Phase 2 180288-69-1
22
Everolimus Approved Phase 2 159351-69-6 70789204 6442177
23
Bevacizumab Approved, Investigational Phase 2 216974-75-3 135329020
24
Lenograstim Approved, Investigational Phase 2 135968-09-1
25
Erlotinib Approved, Investigational Phase 2 183319-69-9, 183321-74-6 176870
26
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 6473866 445643
27
Nivolumab Approved Phase 2 946414-94-4
28
Fludarabine Approved Phase 2 75607-67-9, 21679-14-1 30751 657237
29
Pancrelipase Approved, Investigational Phase 1, Phase 2 53608-75-6 8519
30
Etoposide Approved Phase 2 33419-42-0 36462
31
Decitabine Approved, Investigational Phase 2 2353-33-5 451668
32
Ipilimumab Approved Phase 2 477202-00-9
33
Melphalan Approved Phase 2 148-82-3 4053 460612
34
Picropodophyllin Approved, Investigational Phase 2 518-28-5, 477-47-4 10607 72435
35
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
36
Mycophenolic acid Approved, Investigational Phase 2 24280-93-1 446541
37
Dexrazoxane Approved, Withdrawn Phase 2 24584-09-6 71384
38
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
39
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
40
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
41
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
42
Gemcitabine Approved Phase 1, Phase 2 95058-81-4, 122111-03-9 60750
43
Docetaxel Approved, Investigational Phase 1, Phase 2 114977-28-5 148124
44
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 1, Phase 2 133585-56-5, 60-18-4, 556-02-5 1153 6057
45
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 2 63-91-2, 673-06-3 6140 71567
46
D-Threonine Approved, Experimental, Nutraceutical Phase 2 72-19-5, 632-20-2 205 6288
47
Lorvotuzumab mertansine Investigational Phase 2 1008106-64-6
48
Ado-Trastuzumab Emtansine Phase 2
49
Maytansine Phase 2
50 Angiogenesis Inhibitors Phase 2

Interventional clinical trials:

(show top 50) (show all 54)
# Name Status NCT ID Phase Drugs
1 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Completed 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 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Unknown status NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
5 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
6 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
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 Sorafenib and Dacarbazine in Soft Tissue Sarcoma Completed NCT00837148 Phase 2 Sorafenib and Dacarbazine
9 A Phase II Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
10 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
11 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 Completed NCT02452554 Phase 2
12 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
13 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
14 Phase II Study of MLN8237 in Advanced/Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
15 U.S./Canada Sarcoma Intergroup Study of OSI-774 in Malignant Peripheral Nerve Sheath Tumors, Phase II Completed NCT00068367 Phase 2 erlotinib hydrochloride
16 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
17 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
18 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
19 A Phase 1/2 Multiple Expansion Cohort Trial of MRTX1719 in Patients With Advanced Solid Tumors With MTAP Homozygous Deletion Recruiting NCT05245500 Phase 1, Phase 2 MRTX1719
20 A Phase II Study of ASTX727 in Patients With PRC2 Loss Malignant Peripheral Nerve Sheath Tumor (MPNST) Recruiting NCT04872543 Phase 2 ASTX727
21 A Phase Ib/II Study of APG-115 in Combination With Pembrolizumab in Patients With Unresectable or Metastatic Melanomas or Advanced Solid Tumors Recruiting NCT03611868 Phase 1, Phase 2 APG-115+Pembrolizumab
22 Phase 2 Study Using Tazemetostat in Patients With Recurrent/Refractory and/or Metastatic Malignant Peripheral Nerve Sheath Tumors (MPNST) Recruiting NCT04917042 Phase 2 Tazemetostat
23 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
24 Phase Ib Clinical Trial to Evaluate the Safety and Efficacy of AL2846 Capsules in Chinese Patients With Type I Neurofibromatosis (Neurofibromatosis and Malignant Peripheral Nerve Sheath Tumors) Recruiting NCT05011019 Phase 1, Phase 2 AL2846 capsules
25 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
26 A Non-Inferiority Study of Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Active, not recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin
27 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 Active, not recruiting NCT03872427 Phase 2 Telaglenastat Hydrochloride
28 Hypofractionated Radiotherapy With Hyperthermia in Unresectable or Marginally Resectable Soft Tissue Sarcomas Active, not recruiting NCT03989596 Phase 2
29 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 Active, not recruiting NCT03433183 Phase 2 Selumetinib;Sirolimus
30 A Phase 1/2 Open Label, Basket Study to Assess the Safety, Tolerability and Anti-Tumor Activity of Afamitresgene Autoleucel in Pediatric Subjects With MAGE-A4 Positive Tumors Not yet recruiting NCT05642455 Phase 1, Phase 2
31 A Phase II Study of Pembrolizumab in Patients With Malignant Peripheral Nerve Sheath Tumor (MPNST), Not Eligible for Curative Surgery Terminated NCT02691026 Phase 2 Pembrolizumab
32 A Phase I/Randomized Phase II Study of MLN0128 (TAK-228) VS. Pazopanib in Patients With Locally Advanced/Unresectable and/or Metastatic Sarcoma Terminated NCT02601209 Phase 1, Phase 2 Pazopanib;Pazopanib Hydrochloride;Sapanisertib
33 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
34 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
35 A Phase 1 Study of Doxorubicin and A12 in Advanced Soft Tissue Sarcoma Completed NCT00720174 Phase 1 Doxorubicin Hydrochloride
36 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
37 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
38 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
39 Phase I Study of B7H3 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT04483778 Phase 1
40 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
41 B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR) Recruiting NCT04897321 Phase 1 Fludarabine;Cyclophosphamide;MESNA;B7-H3 CAR T cells
42 A Phase 1 Study of Metronomic Selinexor in Select Soft Tissue Sarcomas and Split Dosing of Selinexor in All Soft Tissue Sarcomas Recruiting NCT04811196 Phase 1 Selinexor
43 Phase I Study of EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT03618381 Phase 1
44 Neoadjuvant Nivolumab Plus Ipilimumab for Newly Diagnosed Malignant Peripheral Nerve Sheath Tumor Recruiting NCT04465643 Phase 1 Nivolumab;Ipilimumab
45 A Phase 1B Study of Ribociclib in Combination With Doxorubicin in Advanced Soft Tissue Sarcomas Active, not recruiting NCT03009201 Phase 1 Doxorubicin Hydrochloride;Ribociclib
46 A Phase I Study of Nivolumab and Intratumoral BO 112 for Resectable Soft Tissue Sarcoma Active, not recruiting NCT04420975 Phase 1
47 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
48 Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis Unknown status NCT01777451
49 Observational - Potentially Actionable Mutations in Archived Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) Completed NCT01567046
50 Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors Recruiting NCT03141021

Search NIH Clinical Center for Neurofibrosarcoma

Cochrane evidence based reviews: neurofibrosarcoma

Genetic Tests for Neurofibrosarcoma

Anatomical Context for Neurofibrosarcoma

Organs/tissues related to Neurofibrosarcoma:

MalaCards : T Cells, Small Intestine, Breast, Lymph Node, Adrenal Gland, Heart, Bone

Publications for Neurofibrosarcoma

Articles related to Neurofibrosarcoma:

(show top 50) (show all 399)
# Title Authors PMID Year
1
Mxi1 mutations in human neurofibrosarcomas. 62 5
10470286 1999
2
Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis. 2: A case report and review of the literature. 53 62
14692188 2003
3
Scalp Plexiform Neurofibrosarcoma With Intrathoracic Fibrosarcoma: A Case Report. 62
36110436 2022
4
Surgery of neurogenic tumors of the sacrum. 62
35248299 2022
5
Epidemiology, Characteristic, and Prognostic Factors of Primary Sporadic Intradural Malignant Peripheral Nerve Sheath Tumor in the Spinal Canal: A Systematic Literature Review. 62
35898898 2022
6
Intracardiac malignant nonchromaffin paraganglioma (chemodectoma) in a cat. 62
34399378 2021
7
Gastrointestinal Stromal Tumor Associated with Neurofibromatosis Type 1 Simulating a Neurofibrosarcoma in a Black African Adult Patient. 62
34720831 2021
8
Mobilization of the neurodynamic system using proprioceptive neuromuscular facilitation decreases pain and increases mobility in lower extremities and Spine-A case report. 62
34391307 2021
9
Neurofibrosarcoma Revisited: An Institutional Case Series of Uterine Sarcomas Harboring Kinase-related Fusions With Report of a Novel FGFR1-TACC1 Fusion. 62
33481389 2021
10
Clinical Outcomes of Surgical Management of Primary Brachial Plexus Tumors. 62
34239232 2021
11
Surgery of neurogenic tumors of the sacrum. 62
33069587 2020
12
Molecular targets for NF1-associated malignant peripheral nerve sheath tumor. 62
32494228 2020
13
A Novel Treatment Modality for Malignant Peripheral Nerve Sheath Tumor Using a Dual-Effect Liposome to Combine Photodynamic Therapy and Chemotherapy. 62
32252313 2020
14
Multiple neurofibromas plus fibrosarcoma with familial NF1 pathogenicity: A case report. 62
32337207 2020
15
Neurofibrosarcoma of the mandible derived from neurofibromatosis. 62
31641652 2019
16
NTRK-1 fusion in endocervical fibroblastic malignant peripheral nerve sheath tumor marking eligibility for larotrectinib therapy: A case report. 62
31080864 2019
17
Giant Pelvic Neurofibroma in Patient with Plexiform Sciatic Neurofibroma and Neurofibromatosis Type 1. 62
31110582 2019
18
Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report. 62
30617438 2019
19
Anterior mediastinal neurofibrosarcoma-a rare manifestation of neurofibromatosis type-1. 62
33060927 2018
20
A case report of cervical neurofibrosarcoma: Clinical presentation, treatment and outcome. 62
29253810 2018
21
Unusual Evolution of Plexiform Neurofibroma in the Scalp: A Case Report. 62
29651404 2018
22
Hemisacrectomy with preservation of the contralateral sacral nerve roots and sacroiliac joint for pelvic neurofibrosarcoma in a 7-year-old child: case report with 2-year follow-up. 62
27689246 2017
23
Doxycycline potentiates antitumor effect of 5-aminolevulinic acid-mediated photodynamic therapy in malignant peripheral nerve sheath tumor cells. 62
28558025 2017
24
Surgical management of parapharyngeal space tumors: The role of cervical and lateral skull base approaches. 62
27929599 2016
25
Seriously saRComa: Neurofibrosarcoma. 62
27789422 2016
26
[Fillet flap for axillary reconstruction]. 62
26195011 2016
27
Malignant peripheral nerve sheath tumor of proximal third tibia. 62
26468455 2015
28
Neurofibrosarcoma of the Subglottis-A Rare Case Report. 62
26405416 2015
29
Neurofibrosarcoma of the lumbar spine with a misleading onset. 62
25542749 2015
30
An unusual oral manifestation of type 1 neurofibromatosis: A case report and review of literature. 62
25810680 2015
31
Malignant peripheral nerve sheath tumor of the third eyelid in a 3-year-old Rhodesian Ridgeback. 62
25678975 2015
32
Abdominal and pelvic tumors with musculoskeletal histology. 62
24629660 2014
33
Imaging of the porta hepatis: spectrum of disease. 62
24428283 2014
34
Value of porous titanium alloy plates for chest wall reconstruction after resection of chest wall tumors. 62
24969880 2014
35
Would a Massive Intra-abdominal Malignant Peripheral Nerve Sheath Tumor with Growth into the Inguinal Canal and Scrotum Preclude Surgical Option? A Case Report and Review of Literature. 62
24465112 2013
36
Neurofibrosarcoma of the gallbladder: a case report. 62
23938157 2013
37
Acute pancreatitis induced by anticancer chemotherapy. 62
24455804 2013
38
Vulvar malignancy in neurofibromatosis syndrome. 62
24167749 2013
39
Giant malignant peripheral nerve sheath tumor of the scalp. 62
23196734 2012
40
[Metastatic neurofibrosarcoma]. 62
21737106 2012
41
Molecular characteristics and pathogenicity of an avian leukosis virus isolated from avian neurofibrosarcoma. 62
22545526 2012
42
Primary intraosseous malignant peripheral nerve sheath tumor of spine with a giant paraspinal and retrospinal subcutaneous extension. 62
23372973 2012
43
Coexistent malignant peripheral nerve sheath tumor and lateral spinal meningoceles. 62
23066463 2012
44
Surgical strategy for presacral tumors: analysis of 33 cases. 62
22340347 2011
45
[Orthopaedic manifestations of Von Recklinghausen's neurofibromatosis]. 62
21074351 2011
46
[The presentation of laryngeal malignant tumors of mesenchymal origin]. 62
21735663 2011
47
Establishment and characterization of a canine soft tissue sarcoma cell line. 62
20974873 2011
48
Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. 62
21317712 2011
49
Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. 62
20399579 2010
50
Could an osteoinductor result in degeneration of a neurofibroma in NF1? 62
20449613 2010

Variations for Neurofibrosarcoma

ClinVar genetic disease variations for Neurofibrosarcoma:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 MXI1 NM_130439.3(MXI1):c.362C>T (p.Ala121Val) SNV Pathogenic
9537 rs137852604 GRCh37: 10:111988034-111988034
GRCh38: 10:110228276-110228276

Expression for Neurofibrosarcoma

Search GEO for disease gene expression data for Neurofibrosarcoma.

Pathways for Neurofibrosarcoma

GO Terms for Neurofibrosarcoma

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell population proliferation GO:0008285 10.13 TP73 TP53 NF2 NF1 CDKN2A
2 negative regulation of protein kinase activity GO:0006469 9.93 NF2 NF1 CDKN2A
3 stem cell proliferation GO:0072089 9.91 TP53 NF1 CD34
4 oligodendrocyte differentiation GO:0048709 9.88 NF1 SOX10 SUZ12
5 positive regulation of phospholipase C activity GO:0010863 9.85 KIT FGFR1
6 regulation of cell population proliferation GO:0042127 9.85 TP53 NF2 NF1 KIT
7 negative regulation of neuroblast proliferation GO:0007406 9.8 NF1 TP53
8 neuroblast proliferation GO:0007405 9.8 TP53 SOX10 NF1
9 cell population proliferation GO:0008283 9.7 TP53 TACC1 SUZ12 NF1 KIT
10 somatic stem cell division GO:0048103 9.69 KIT CDKN2A
11 mast cell proliferation GO:0070662 9.67 NF1 KIT
12 negative regulation of glial cell proliferation GO:0060253 9.65 NF1 TP53
13 Schwann cell proliferation GO:0014010 9.56 NF2 NF1
14 negative regulation of cell-matrix adhesion GO:0001953 9.43 NF2 NF1 CDKN2A
15 negative regulation of Schwann cell proliferation GO:0010626 9.1 SOX10 NF2 NF1

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

# Name GO ID Score Top Affiliating Genes
1 promoter-specific chromatin binding GO:1990841 9.43 TP53 SUZ12 SOX10
2 RNA polymerase II-specific DNA-binding transcription factor binding GO:0061629 9.17 TP73 TP53 CDKN2A CD34

Sources for Neurofibrosarcoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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