MCID: NRF003
MIFTS: 42

Neurofibrosarcoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Neurofibrosarcoma

MalaCards integrated aliases for Neurofibrosarcoma:

Name: Neurofibrosarcoma 12 74 52 54 6 43 15 71
Malignant Peripheral Nerve Sheath Tumor 71
Neurosarcoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3512
MeSH 43 D018319
SNOMED-CT 67 19897006
UMLS 71 C0206729 C0751690

Summaries for Neurofibrosarcoma

MalaCards based summary : Neurofibrosarcoma, also known as malignant peripheral nerve sheath tumor, is related to adult 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 Glioblastoma Multiforme and TP53 Network. The drugs Mechlorethamine and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include breast, bone and small intestine, and related phenotypes are Increased proliferation and cellular

Wikipedia : 74 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 275)
# Related Disease Score Top Affiliating Genes
1 adult malignant schwannoma 31.7 TP53 NF2 NF1 CD34
2 malignant peripheral nerve sheath tumor 31.7 TP53 S100A1 NF2 NF1 KIT CD34
3 neurofibromatosis, type i 31.2 NF1 KIT
4 neurofibromatosis, type iv, of riccardi 30.6 TP53 NFIC NF2 NF1 KIT
5 spinal meningioma 30.6 NF2 NF1
6 rare tumor 30.5 NF2 KIT
7 meckel diverticulum 30.1 KIT CD34
8 lipomatosis, multiple 29.9 NF1 KIT CD34
9 thymoma 29.8 TP53 KIT ENO2
10 neuroma 29.8 NF2 NF1 ENO2 CD34
11 hemangiopericytoma, malignant 29.8 KIT ENO2 CD34
12 sarcoma, synovial 29.8 NF1 KIT CD34
13 neurofibroma 29.8 TP53 S100A1 NF2 NF1 KIT CD34
14 angiosarcoma 29.7 TP53 KIT CD34
15 leiomyosarcoma 29.5 TP53 S100A1 NF1 KIT ENO2
16 neurilemmoma 29.5 S100A1 NF2 NF1 KIT ENO2 CD34
17 sarcoma 29.5 TP53 NF2 KIT FES ENO2
18 mesenchymal cell neoplasm 29.5 TP53 NF1 KIT CD34
19 histiocytoma 29.4 TP53 KIT ENO2 CD34
20 hemangioma 29.4 TP53 KIT ENO2 CD34
21 connective tissue benign neoplasm 29.3 TP53 NF1 KIT ENO2 CD34
22 plexiform neurofibroma 29.1 TP53 NFIC NF2 NF1 KIT CD34
23 meningioma, familial 28.8 TP53 NF2 NF1 KIT ENO2 CD34
24 childhood malignant schwannoma 11.2
25 amyloid tumor 10.5 NF2 NF1
26 neurilemmoma of the fifth cranial nerve 10.5 NF2 NF1
27 trigeminal nerve neoplasm 10.5 NF2 NF1
28 peripheral nerve schwannoma 10.5 NF2 NF1
29 polyp of corpus uteri 10.5 NF2 NF1
30 rete ovarii adenoma 10.5 NF2 NF1
31 rete ovarii neoplasm 10.5 NF2 NF1
32 spinal canal and spinal cord meningioma 10.5 NF2 NF1
33 optic nerve astrocytoma 10.4 NF1 ENO2
34 schwannoma of twelfth cranial nerve 10.4 NF2 NF1
35 skin granular cell tumor 10.4 NF1 ENO2
36 hypoglossal nerve disease 10.4 NF2 NF1
37 corpus callosum lipoma 10.4 NF2 NF1
38 meningothelial meningioma 10.4 NF2 ENO2
39 rhabdoid meningioma 10.4 NF2 ENO2
40 cerebral hemisphere lipoma 10.4 NF2 NF1
41 bile duct mucinous adenocarcinoma 10.4 TP53 ENO2
42 central nervous system hemangioma 10.4 NF1 CD34
43 frontal lobe neoplasm 10.4 TP53 ENO2
44 lung meningioma 10.3 NF2 CD34
45 intraneural perineurioma 10.3 S100A1 NF2
46 parasagittal meningioma 10.3 NF2 CD34
47 polyploidy 10.3 TP73 TP53
48 papillary tumor of the pineal region 10.3 S100A1 ENO2
49 cerebral convexity meningioma 10.3 NF2 CD34
50 wilms tumor predisposition 10.3 TP53 NF1

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:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.62 NF2 TP53

MGI Mouse Phenotypes related to Neurofibrosarcoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.13 CD34 DCK ENO2 KIT MXD3 MXI1
2 craniofacial MP:0005382 10.01 ENO2 FES KIT NF1 NF2 NFIC
3 endocrine/exocrine gland MP:0005379 9.97 DCK FES KIT MXI1 NF1 NF2
4 immune system MP:0005387 9.91 CD34 DCK FES KIT MXI1 NF1
5 neoplasm MP:0002006 9.76 CD34 FES KIT MXI1 NF1 NF2
6 hearing/vestibular/ear MP:0005377 9.72 KIT NF1 NF2 TP53 TP73
7 reproductive system MP:0005389 9.56 FES KIT MXI1 NF1 NF2 NFIC
8 respiratory system MP:0005388 9.17 ENO2 KIT NF1 NF2 S100A1 TP53

Drugs & Therapeutics for Neurofibrosarcoma

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

(show top 50) (show all 102)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
2
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
3
Lenograstim Approved, Investigational Phase 3 135968-09-1
4
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
5
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
6
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
7
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
8
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
9
Etoposide Approved Phase 3 33419-42-0 36462
10
Epirubicin Approved Phase 3 56420-45-2 41867
11
Dacarbazine Approved, Investigational Phase 3 4342-03-4 5351166
12
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
13
Trabectedin Approved, Investigational Phase 2, Phase 3 114899-77-3 108150
14
Histamine Approved, Investigational Phase 3 51-45-6 774
15
Cyproheptadine Approved Phase 3 129-03-3 2913
16 Antimitotic Agents Phase 3
17 Immunologic Factors Phase 3
18 Anti-Infective Agents Phase 3
19 Immunosuppressive Agents Phase 3
20
Isophosphamide mustard Phase 2, Phase 3 0
21 Antirheumatic Agents Phase 3
22 Etoposide phosphate Phase 3
23 Antibiotics, Antitubercular Phase 2, Phase 3
24 Anti-Bacterial Agents Phase 2, Phase 3
25 Protein Kinase Inhibitors Phase 2, Phase 3
26 Neurotransmitter Agents Phase 3
27 Alkylating Agents Phase 2, Phase 3
28 Topoisomerase Inhibitors Phase 2, Phase 3
29
Liposomal doxorubicin Phase 2, Phase 3 31703
30 Imatinib Mesylate Phase 2, Phase 3 220127-57-1 123596
31 Gastrointestinal Agents Phase 3
32 Dermatologic Agents Phase 3
33 Antipruritics Phase 3
34 Histamine H1 Antagonists Phase 3
35 Serotonin Agents Phase 3
36 Histamine Antagonists Phase 3
37
Histamine Phosphate Phase 3 51-74-1 65513
38 Serotonin Antagonists Phase 3
39 Anti-Allergic Agents Phase 3
40
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
41
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
42
Bevacizumab Approved, Investigational Phase 2 216974-75-3
43
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
44
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
45
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
46
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
47
Xylometazoline Approved, Investigational Phase 1, Phase 2 526-36-3 5709
48
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
49
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
50
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189

Interventional clinical trials:

(show top 50) (show all 80)
# Name Status NCT ID Phase Drugs
1 Localized Non-Rhabdomyosarcoma Soft Tissue Sarcomas Unknown status NCT00334854 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Randomised Trial of Post-Operative Radiotherapy Given to Adult Patients With Extremity Soft Tissue Sarcoma [VORTEX] Completed NCT00423618 Phase 3
3 Randomised Trial Of Single Agent Doxorubicin Versus Doxorubicin Plus Ifosfamide In The First Line Treatment Of Advanced Or Metastatic Soft Tissue Sarcoma Completed NCT00061984 Phase 3 doxorubicin hydrochloride;ifosfamide
4 A Phase III Randomized Study of Preoperative Radiation Plus Surgery Versus Surgery Alone for Patients With Retroperitoneal Sarcomas (RPS) Completed NCT00091351 Phase 3
5 Randomized Phase III Trial of Two Investigational Schedules of Ifosfamide vs. Standard Dose Doxorubicin in Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00003212 Phase 3 doxorubicin hydrochloride;ifosfamide
6 Randomized Study Comparing Neoadjuvant Chemotherapy Etoposide + Ifosfamide + Adriamycin (EIA) Combined With Regional Hyperthermia (RHT) Versus Neoadjuvant Chemotherapy Alone in the Treatment of High-Risk Soft Tissue Sarcomas in Adults Completed NCT00003052 Phase 3 doxorubicin hydrochloride;etoposide;ifosfamide
7 METASTASECTOMY AND CHEMOTHERAPY FOR LUNG METASTASES FROM SOFT TISSUE SARCOMA: A RANDOMIZED PHASE III STUDY (AN INTERGROUP STUDY WITH THE SCANDINAVIAN SARCOMA GROUP) Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
8 RANDOMISED TRIAL OF ADJUVANT CHEMOTHERAPY WITH HIGH-DOSE DOXORUBICIN, IFOSFAMIDE AND LENOGRASTIM IN HIGH GRADE SOFT TISSUE SARCOMA Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
9 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) Completed NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
10 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
11 MMT 95 Study For Rhabdomyosarcoma and Other Malignant Soft Tissue Tumors of Childhood Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
12 Localized High-Risk Soft Tissue Sarcomas Of The Extremities And Trunk Wall In Adults: An Integrating Approach Comprising Standard Vs Histotype-Tailored Neoadjuvant Chemotherapy Recruiting NCT01710176 Phase 3 epirubicin 60 mg/m2/day (days 1, 2) and ifosfamide 3 g/m2/day (days 1, 2, 3);gemcitabine 900 mg/m2 (days 1 and 8) and docetaxel 75 mg/m2 (day 8);trabectedin 1.3 mg/m2;high-dose ifosfamide 14 g/m2, given in in 14 days;etoposide 150 mg/m2/day (days 1, 2, 3) and ifosfamide 3g/m2/day (days 1, 2, 3);gemcitabine 1800 mg/m2 (day 1) and dacarbazine 500 mg/m2 (day 1)
13 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
14 TRUSTS: A Phase IIB/III Multicenter Study Comparing the Efficacy of TRabectedin Administered as a 3-Hour or 24-Hour Infusion to Doxorubicin in Patients With Advanced or Metastatic Untreated Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3 doxorubicin hydrochloride;trabectedin
15 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
16 Phase II Evaluation of Temodal (Temozolomide, Schering) in Previously Treated Advanced Sarcomas Unknown status NCT00003718 Phase 2 temozolomide
17 Randomized Phase II Study of Brostallicin (PNU-166196A) Versus Doxorubicin as First Line Chemotherapy in Patients With Advanced or Metastatic Soft Tissue Sarcoma Unknown status NCT00410462 Phase 2 brostallicin;doxorubicin hydrochloride
18 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
19 U.S./Canada Sarcoma Intergroup Study of OSI-774 in Malignant Peripheral Nerve Sheath Tumors, Phase II Completed NCT00068367 Phase 2 erlotinib hydrochloride
20 A Phase II Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
21 Phase II Study of MLN8237 in Advanced/Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
22 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
23 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
24 Phase II Trial of Ixabepilone (BMS-247550), an Epothilone B Analog, in Children and Young Adults With Refractory Solid Tumors Completed NCT00331643 Phase 2 ixabepilone
25 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
26 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
27 Exatecan As Second-Line Treatment In Advanced Adult Soft Tissue Sarcoma: A Phase II - Study Of The EORTC Soft Tissue And Bone Sarcoma Group Completed NCT00041236 Phase 2 exatecan mesylate
28 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
29 Phase II Study on ET-743 in Advanced Soft Tissue Sarcomas of the Adult Completed NCT00003939 Phase 2 trabectedin
30 Phase II Trial of Sorafenib and Dacarbazine in Soft Tissue Sarcoma Completed NCT00837148 Phase 2 Sorafenib and Dacarbazine
31 A Phase II Study Of Perifosine (D-21266) In Patients With Previously Untreated Metastatic Or Locally Advanced Soft Tissue Sarcoma Completed NCT00053794 Phase 2 perifosine
32 Efficacy And Safety Study Of Brostallicin In Patients With Locally Advanced Or Metastatic Soft Tissue Sarcoma Failing One Prior Chemotherapy Treatment Completed NCT00041249 Phase 2 brostallicin
33 Phase II Trial of Gleevec (Formerly Known as STI571) in Patients With Soft Tissue and Bone Sarcomas: A Multi-Disciplinary Trial of the North American Sarcoma Study Group of the Connective Tissue Oncology Society Completed NCT00031915 Phase 2 imatinib mesylate
34 Multicenter Parallel Phase II Trial of BI 2536 Administered as One Hour IV Infusion Every 3 Weeks in Defined Cohorts of Patients With Various Solid Tumors. A New Drug Screening Program of the EORTC Network of Core Institutions (NOCI) Completed NCT00526149 Phase 2 BI 2536
35 Dose Finding and Phase II Study of STI 571 in Advanced Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
36 A Multicenter Phase II Study of Continuous Dosing of Sunitinib (Sutent®, SU11248) in Non-GIST Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
37 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
38 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
39 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
40 A Phase II Trial Evaluating APX005M (a CD40 Agonistic Monoclonal Antibody) in Combination With Standard-of-Care Doxorubicin for the Treatment of Advanced Sarcomas Recruiting NCT03719430 Phase 2 Doxorubicin;APX005M
41 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
42 Phase I/II Trial of PLX3397 in Children and Young Adults With Refractory Leukemias and Refractory Solid Tumors Including Neurofibromatosis Type 1 (NF1) Associated Plexiform Neurofibromas (PN) Recruiting NCT02390752 Phase 1, Phase 2 PLX3397
43 A Phase II Study of Cabozantinib (XL184) for Plexiform Neurofibromas in Subjects With Neurofibromatosis Type 1 in Children and Adults Recruiting NCT02101736 Phase 2 Cabozantinib
44 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
45 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
46 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
47 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Primary Unresectable or Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Recruiting NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
48 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 Glutaminase Inhibitor CB-839 Hydrochloride
49 Preoperative Hypofractionated Radiotherapy With Hyperthermia in Unresectable or Marginally Resectable Soft Tissue Sarcomas Recruiting NCT03989596 Phase 2
50 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2

Search NIH Clinical Center for Neurofibrosarcoma

Cochrane evidence based reviews: neurofibrosarcoma

Genetic Tests for Neurofibrosarcoma

Anatomical Context for Neurofibrosarcoma

MalaCards organs/tissues related to Neurofibrosarcoma:

40
Breast, Bone, Small Intestine, Lung, Heart, Skin, Prostate

Publications for Neurofibrosarcoma

Articles related to Neurofibrosarcoma:

(show top 50) (show all 386)
# Title Authors PMID Year
1
Mxi1 mutations in human neurofibrosarcomas. 61 6
10470286 1999
2
Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis. 2: A case report and review of the literature. 54 61
14692188 2003
3
Neurofibrosarcoma of the mandible derived from neurofibromatosis. 61
31641652 2019
4
NTRK-1 fusion in endocervical fibroblastic malignant peripheral nerve sheath tumor marking eligibility for larotrectinib therapy: A case report. 61
31080864 2019
5
Giant Pelvic Neurofibroma in Patient with Plexiform Sciatic Neurofibroma and Neurofibromatosis Type 1. 61
31110582 2019
6
Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report. 61
30617438 2019
7
Unusual Evolution of Plexiform Neurofibroma in the Scalp: A Case Report. 61
29651404 2018
8
A case report of cervical neurofibrosarcoma: Clinical presentation, treatment and outcome. 61
29253810 2018
9
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. 61
27689246 2017
10
Doxycycline potentiates antitumor effect of 5-aminolevulinic acid-mediated photodynamic therapy in malignant peripheral nerve sheath tumor cells. 61
28558025 2017
11
Surgical management of parapharyngeal space tumors: The role of cervical and lateral skull base approaches. 61
27929599 2016
12
Seriously saRComa: Neurofibrosarcoma. 61
27789422 2016
13
[Fillet flap for axillary reconstruction]. 61
26195011 2016
14
Malignant peripheral nerve sheath tumor of proximal third tibia. 61
26468455 2015
15
Neurofibrosarcoma of the Subglottis-A Rare Case Report. 61
26405416 2015
16
Neurofibrosarcoma of the lumbar spine with a misleading onset. 61
25542749 2015
17
Malignant peripheral nerve sheath tumor of the third eyelid in a 3-year-old Rhodesian Ridgeback. 61
25678975 2015
18
An unusual oral manifestation of type 1 neurofibromatosis: A case report and review of literature. 61
25810680 2015
19
Abdominal and pelvic tumors with musculoskeletal histology. 61
24629660 2014
20
Imaging of the porta hepatis: spectrum of disease. 61
24428283 2014
21
Value of porous titanium alloy plates for chest wall reconstruction after resection of chest wall tumors. 61
24969880 2014
22
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. 61
24465112 2013
23
Neurofibrosarcoma of the gallbladder: a case report. 61
23938157 2013
24
Acute pancreatitis induced by anticancer chemotherapy. 61
24455804 2013
25
Vulvar malignancy in neurofibromatosis syndrome. 61
24167749 2013
26
Giant malignant peripheral nerve sheath tumor of the scalp. 61
23196734 2012
27
[Metastatic neurofibrosarcoma]. 61
21737106 2012
28
Molecular characteristics and pathogenicity of an avian leukosis virus isolated from avian neurofibrosarcoma. 61
22545526 2012
29
Primary intraosseous malignant peripheral nerve sheath tumor of spine with a giant paraspinal and retrospinal subcutaneous extension. 61
23372973 2012
30
Coexistent malignant peripheral nerve sheath tumor and lateral spinal meningoceles. 61
23066463 2012
31
Surgical strategy for presacral tumors: analysis of 33 cases. 61
22340347 2011
32
[Orthopaedic manifestations of Von Recklinghausen's neurofibromatosis]. 61
21074351 2011
33
[The presentation of laryngeal malignant tumors of mesenchymal origin]. 61
21735663 2011
34
Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. 61
21317712 2011
35
Establishment and characterization of a canine soft tissue sarcoma cell line. 61
20974873 2011
36
Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. 61
20399579 2010
37
Could an osteoinductor result in degeneration of a neurofibroma in NF1? 61
20449613 2010
38
Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases. 61
19467792 2010
39
[Spontaneous hemothorax in a neurofibromatosis type I patient - a case report]. 61
19649550 2009
40
[Diagnosis and surgical therapy of primary retroperitoneal neurogenic tumors]. 61
19953887 2009
41
Rare association of Hodgkin lymphoma, Graves' disease and myasthenia gravis complicated by post-radiation neurofibrosarcoma: coincidence or genetic susceptibility? 61
19381762 2009
42
Sternal resection and reconstruction after malignant tumours. 61
19211374 2009
43
Non-germ cell tumours arising in germ cell tumours (teratoma with malignant transformation) in men: CT and MR findings. 61
19056194 2009
44
Loss of heterozygosity (LOH)--implications for human genetic identification. 61
19419947 2009
45
Penile and preputial tumours in the horse: a retrospective study of 114 affected horses. 61
18487101 2008
46
[Results of surgical management of spinal neurinomas and neurofibromas at Yaounde]. 61
19472736 2008
47
Gross and histopathological characteristics of two lipomas and a neurofibrosarcoma detected in aquacultured southern bluefin tuna, Thunnus maccoyii (Castelnau), in South Australia. 61
18353015 2008
48
[Neurofibrosarcoma with pulmonary metastasis in patient with neurofibromatosis type I]. 61
18560687 2008
49
A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1. 61
27303156 2008
50
[Cervicobrachial neuralgia revealing neurosarcoma]. 61
18342062 2008

Variations for Neurofibrosarcoma

ClinVar genetic disease variations for Neurofibrosarcoma:

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

Expression for Neurofibrosarcoma

Search GEO for disease gene expression data for Neurofibrosarcoma.

Pathways for Neurofibrosarcoma

Pathways related to Neurofibrosarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.84 TP53 NF2 NF1
2
Show member pathways
10.3 TP73 TP53

GO Terms for Neurofibrosarcoma

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 9.62 TP53 NF2 NF1 MXI1
2 negative regulation of transcription by RNA polymerase II GO:0000122 9.55 TP53 S100A1 NFIC MXI1 MXD3
3 negative regulation of cell-matrix adhesion GO:0001953 9.26 NF2 NF1
4 regulation of cell proliferation GO:0042127 9.02 TP53 NF2 NF1 KIT FES
5 negative regulation of neuroblast proliferation GO:0007406 8.96 TP53 NF1

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

# Name GO ID Score Top Affiliating Genes
1 MDM2/MDM4 family protein binding GO:0097371 8.62 TP73 TP53

Sources for Neurofibrosarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
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48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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