MPNST
MCID: MLG077
MIFTS: 53

Malignant Peripheral Nerve Sheath Tumor (MPNST) malady

Neuronal, Cancer categories

Summaries for Malignant Peripheral Nerve Sheath Tumor

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 A malignant peripheral nerve sheath tumor (mpnst) is a tumor that develops from nerve tissue.  the first symptom of mpnst is 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. last updated: 10/18/2011

MalaCards: Malignant Peripheral Nerve Sheath Tumor, also known as MPNST, is related to neurofibromatosis and plexiform neurofibroma. An important gene associated with Malignant Peripheral Nerve Sheath Tumor is S100B (S100 calcium binding protein B), and among its related pathways are GPCR Pathway and Glioblastoma Multiforme. The compounds estrogen and glucose have been mentioned in the context of this disorder. Related mouse phenotypes are limbs/digits/tail and embryogenesis.

Wikipedia:64 A malignant peripheral nerve sheath tumor (MPNST) (also known as \"Malignant schwannoma,\"... more...

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 49Orphanet, 61UMLS, 57SNOMED-CT, 35MeSH, 40NCIt
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Neuronal


Characteristics (Orphanet epidemiological data):

49
malignant peripheral nerve sheath tumor:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

malignant peripheral nerve sheath tumor 8 43 10 49 61
mpnst 43 45 49
malignant neoplasm of the peripheral nerve sheath 8
malignant peripheral nerve sheath tumors 45
malignant neurofibroma 49
malignant neurilemmoma 49
malignant schwannoma 49
neurogenic sarcoma 49
neurofibrosarcoma 49


External Ids:

Disease Ontology8 DOID:5940
MeSH35 D018317
NCIt40 C3798

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Malignant Peripheral Nerve Sheath Tumor family:

benign peripheral nerve sheath tumor

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

(show top 50)    (show all 213)
idRelated DiseaseScoreTop Affiliating Genes
1neurofibromatosis31.8KIT, MMP13, NGFR, NF1, NF2, PDGFRA
2plexiform neurofibroma31.1MMP13, NF1, PDGFRA
3synovial sarcoma31.1CALB2, S100B, NES, NF1, MMP13, KIT
4ganglioneuroma31.0S100B, CHGA, NGFR
5neurofibrosarcoma30.9NF2, NF1
6angiosarcoma30.7PDGFRA, CALB2
7pheochromocytoma30.7NGFR, NF1, CHGA, CALB2
8gastrointestinal stromal tumor30.7KIT, PDGFRA
9smooth muscle tumor30.5KIT, S100A1, S100B
10papillary carcinoma30.5CHGA, S100A1, S100A6, CALB2
11adenocarcinoma30.5CALB2, S100A6, BMP2, TOP2A, NES, CHGA
12bladder carcinoma30.5MMP13, CDKN2A, NGFR, TOP2A
13rhabdoid tumor29.9SMARCB1
14thymoma29.9CALB2, NGFR
15wilms tumor29.9CALB2, TOP2A, MYOG, SMARCB1
16epithelioid malignant peripheral nerve sheath tumor11.0
17malignant triton tumor10.8
18benign peripheral nerve sheath tumor10.8
19malignant perineurioma10.7
20soft tissue sarcoma10.6
21n syndrome10.5
22small cell sarcoma10.5
23malignant mesenchymal tumor10.5
24benign schwannoma10.5
25malignant cardiac peripheral nerve sheath neoplasm10.3
26malignant oculomotor nerve tumor10.3
27malignant peritoneal mesothelioma10.3
28malignant spindle cell melanoma10.3
29cellular schwannoma10.3
30plexiform schwannoma10.3
31malignant epithelial mesothelioma10.3
32cervix melanoma10.3
33childhood malignant schwannoma10.3
34meningocele10.3
35intussusception10.3
36aortic malignant tumor10.3
37monophasic synovial sarcoma10.3
38fibrous synovial sarcoma10.3
39malignant glandular tumor of peripheral nerve sheath10.3
40spindle cell sarcoma10.3
41melanocytic psammomatous mpnst10.3
42askin's tumor10.3
43mediastinum neurofibroma10.3
44horner's syndrome10.3
45bladder disease10.3
46t cell deficiency10.3
47giant congenital nevus10.3
48carotid body tumor10.3
49desmoplastic small round cell tumor10.3
50heart tumor10.3

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



Diseases related to malignant peripheral nerve sheath tumor

Clinical Features for Malignant Peripheral Nerve Sheath Tumor

Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Malignant Peripheral Nerve Sheath Tumor

Drug clinical trials:

Search ClinicalTrials for Malignant Peripheral Nerve Sheath Tumor

Search NIH Clinical Center for Malignant Peripheral Nerve Sheath Tumor

Search CenterWatch for Malignant Peripheral Nerve Sheath Tumor

Genetic Tests for Malignant Peripheral Nerve Sheath Tumor

Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

Animal Models for Malignant Peripheral Nerve Sheath Tumor or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

37 (show all 22)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537110.7SOX10
2MP:000538010.7SOX10, BMP2, MYOG, NES, NF2
3MP:001077110.7MMP13, CDKN2A, MYOG, PDGFRA, SOX10, S100B
4MP:000118610.7NF1
5MP:000537710.6NF1, NGFR, NF2, BMP2, APOD
6MP:000538810.6PDGFRA, MYOG, NGFR, PRG2, NF2, NF1
7MP:000363110.6NF2, NF1, NGFR, KIT, APOD, SOX10
8MP:000537610.6PDGFRA, MYOG, APOD, NF2, NF1, SMARCB1
9MP:000539010.6PDGFRA, BMP2, NF1, MYOG, MMP13, NF2
10MP:000287310.5CALB2, S100B, NGFR, S100A1, MYOG, KIT
11MP:000537810.5MMP13, SOX10, PDGFRA, NES, CHGA, NF2
12MP:000538610.5S100B, KIT, CALB2, CDKN2A, NGFR, MYOG
13MP:000536910.5NF1, PDGFRA, CDKN2A, NGFR
14MP:000538110.5KIT, CDKN2A, BMP2, PDGFRA, SOX10, NGFR
15MP:000538510.5CHGA, BMP2, PDGFRA, SMARCB1, S100A1, NF1
16MP:001076810.5CHGA, NF2, NES, BMP2, APOD, PDGFRA
17MP:000200610.4PDGFRA, SMARCB1, KIT, CDKN2A, NF1, NF2
18MP:000538410.4SOX10, MMP13, KIT, SMARCB1, NF1, NES
19MP:000537910.3PDGFRA, NF2, NF1, CDKN2A, SOX10, KIT
20MP:000536710.3KIT, PDGFRA, NF2, NF1, NGFR, CHGA
21MP:000538210.2PDGFRA, NF2, BMP2, NF1, NGFR, KIT
22MP:000537010.0SMARCB1, NF1, NGFR, CDKN2A, NF2, KIT

Publications for Malignant Peripheral Nerve Sheath Tumor

Sources:
51PubMed
See all sources

Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50)    (show all 433)
idTitleAuthorsYear
1
Concurrence of malignant peripheral nerve sheath tumor at the site of complex regional pain syndrome type 1 - a case report -. (23614078)
2013
2
Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. (23084090)
2013
3
Malignant peripheral nerve sheath tumor of the chest wall associated with neurofibromatosis: a case report. (23825788)
2013
4
Common genetic variants in the microRNA biogenesis pathway are associated with malignant peripheral nerve sheath tumor risk in a Chinese population. (23763827)
2013
5
Microsurgical management of giant malignant peripheral nerve sheath tumor of the scalp: two case reports and a literature review. (24112233)
2013
6
An unusual case of a malignant peripheral nerve sheath tumor. (23730615)
2013
7
Multifocal spinal malignant peripheral nerve sheath tumor in an immunocompromised individual: case report and review of literature. (24249554)
2013
8
Conventional spindle cell-type malignant peripheral nerve sheath tumor arising in a sporadic schwannoma. (24007690)
2013
9
Serum biomarkers for neurofibromatosis type 1 and early detection of malignant peripheral nerve-sheath tumors. (23618374)
2013
10
Primary malignant peripheral nerve sheath tumor at unusual location. (24174807)
2013
11
Malignant peripheral nerve sheath tumor arising in a traumatic neuroma: a case report. (23701941)
2013
12
Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1. (24343189)
2013
13
Leptomeningeal metastasis of an intradural malignant peripheral nerve sheath tumor. (23664130)
2013
14
Malignant peripheral nerve sheath tumor: pathology and genetics. (24418643)
2013
15
Metastatic malignant peripheral nerve sheath tumor in neurofibromatosis type 1: a geriatric patient report. (23047517)
2012
16
A case of huge malignant peripheral nerve sheath tumor (MPNST) in the back. (23002449)
2012
17
Temporal bone histopathology case of the month: Malignant peripheral nerve sheath tumor arising within vestibular schwannoma. (22935807)
2012
18
Malignant peripheral nerve sheath tumor of the oral cavity. (22871311)
2012
19
Survivin is a viable target for the treatment of malignant peripheral nerve sheath tumors. (22407831)
2012
20
Malignant peripheral nerve sheath tumor in spine: imaging manifestations. (22542380)
2012
21
Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach. (21529197)
2011
22
Malignant peripheral nerve sheath tumor of kidney. (22057067)
2011
23
Perineural extension of cutaneous desmoplastic melanoma mimicking an intracranial malignant peripheral nerve sheath tumor. Case report. (21548747)
2011
24
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. (20952276)
2011
25
Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis Type 1 and middle cerebral artery aneurysm treated with endovascular coil embolization. (21961539)
2011
26
Recurring intracranial malignant peripheral nerve sheath tumor: case report and systematic review of the literature. (21242836)
2011
27
Neuregulin-1 beta and neuregulin-1 alpha differentially affect the migration and invasion of malignant peripheral nerve sheath tumor cells. (19306381)
2009
28
Malignant peripheral nerve sheath tumor of the lumbar spine. (19585001)
2009
29
Malignant peripheral nerve sheath tumor of the hand. (19182591)
2009
30
Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors. (19182148)
2009
31
Malignant peripheral nerve sheath tumor of the orbit: malignant transformation from neurofibroma without neurofibromatosis. (18806670)
2008
32
High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization. (18281533)
2008
33
Induction of apoptosis in neurofibromatosis type 1 malignant peripheral nerve sheath tumor cell lines by a combination of novel farnesyl transferase inhibitors and lovastatin. (18367665)
2008
34
Malignant peripheral nerve sheath tumor in a hamster. (17994674)
2007
35
Malignant peripheral nerve sheath tumor of the occipital region: case report. (18162865)
2007
36
Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy. (16182351)
2006
37
Cervicothoracic malignant peripheral nerve sheath tumor in a 12-year-old girl with neurofibromatosis type 1. (16981098)
2006
38
Malignant peripheral nerve sheath tumor arising from an adrenal ganglioneuroma in a 6-year-old boy. (15022069)
2004
39
Retroperitoneal malignant peripheral nerve sheath tumor: evaluation with serial FDG-PET. (15192465)
2004
40
p15INK4b, p14ARF, and p16INK4a inactivation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. (14519636)
2003
41
Hypertrophic neuropathies and malignant peripheral nerve sheath tumors in transgenic mice overexpressing glial growth factor beta3 in myelinating Schwann cells. (12917360)
2003
42
Malignant peripheral nerve sheath tumor of the vulva. A case report. (12380453)
2002
43
Malignant peripheral nerve sheath tumor with perineurial differentiation: "malignant perineurioma". (12135468)
2002
44
Expression of protein gene product 9.5 in epithelioid and conventional malignant peripheral nerve sheath tumors. (11570907)
2001
45
Pathologic quiz case: deep venous thrombosis in a 21-year-old woman with a retroperitoneal mass. Pathologic diagnosis: malignant peripheral nerve sheath tumor. (11079040)
2000
46
Malignant peripheral nerve sheath tumor with extensive miliary metastases: a case report. (9228261)
1997
47
Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: a case report and review of the literature. (9024521)
1997
48
Purely epithelioid malignant peripheral nerve sheath tumor of the vulva. (9142667)
1997
49
Intraosseous malignant peripheral nerve sheath tumor. Report of a case and review of the literature. (7726730)
1995
50
Malignant peripheral nerve sheath tumor arising spontaneously from retroperitoneal ganglioneuroma: a case report, review of the literature, and immunohistochemical study. (1544674)
1992

Genetic Variations for Malignant Peripheral Nerve Sheath Tumor

Expression for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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

Pathways for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
52QIAGEN
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Pathways related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4PDGFRA, MMP13, NGFR, BMP2
210.4NF2, NF1, CDKN2A, PDGFRA
3
Hide members
10.4BMP2, PRG2, NGFR, KIT, PDGFRA
4
Hide members
10.3KIT, SOX10, PDGFRA, BMP2, PRG2, NF1

Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 60Tocris Bioscience, 2BitterDB
See all sources

Compounds related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 74)
idCompoundScoreTop Affiliating Genes
1estrogen4510.8CALB2, NF1, MYOG
2glucose4510.8NGFR, CDKN2A, MYOG
3forskolin45 50 1112.8MYOG, NGFR
4b 7234510.7S100B, S100A1, CHGA, CALB2
5dopamine45 29 11 2413.7CHGA, NGFR, NF1, NES, BMP2, CALB2
6fatty acid4510.7CDKN2A, NF1, TOP2A, S100A6, MYOG
7mucicarmine4510.7CALB2, S100B, S100A1
8agarose4510.7CDKN2A, S100B, APOD, MMP13, KIT
9silver4510.7CDKN2A, CHGA, S100A1, S100A6, CALB2
10serine4510.7TOP2A, NF1, NF2, S100B, BMP2, MMP13
11paclitaxel45 50 1112.7CDKN2A, CHGA, S100B, TOP2A, CALB2
12hematoxylin4510.7PRG2, S100A1, S100B, CHGA, KIT, CDKN2A
13matrigel4510.7MMP13, CHGA, NES, BMP2, NGFR
14gaba4510.7S100B, NGFR, CHGA, NES, CALB2
15creatinine4510.7BMP2, CHGA, CDKN2A, APOD
16rapamycin4510.7BMP2, NF2, KIT, MYOG, NF1, PDGFRA
17thymidine45 2411.7S100B, TOP2A, PDGFRA, CALB2, BMP2, KIT
18steroid4510.7CALB2, S100A6, BMP2, TOP2A, NF1, MYOG
19acetylcholine45 50 29 11 2414.7MYOG, CHGA, PRG2, NES, CALB2, S100A1
20actinomycin d4510.7MMP13, CDKN2A, PRG2, TOP2A, BMP2, KIT
21oxygen45 2411.7MMP13, CDKN2A, NES, CHGA, MYOG, NGFR
22p0024510.7TOP2A, KIT, CHGA, MMP13
23bromodeoxyuridine4510.6MYOG, CHGA, S100B, NES
24cyclic amp45 2411.6MMP13, CDKN2A, NGFR, NF1, S100B, CHGA
25ribonucleic acid4510.6KIT, BMP2, MMP13, MYOG, CHGA
26choline45 11 2412.6S100B, NGFR, NES, CALB2
27vegf4510.6MMP13, KIT, CHGA, BMP2, CDKN2A, NGFR
28zinc45 2411.6CALB2, S100B, MMP13, S100A6, CHGA, NGFR
29lipid4510.6S100B, BMP2, NES, PRG2, CHGA, NF1
30atp45 2911.6CDKN2A, NF1, CHGA, PRG2, TOP2A, PDGFRA
31dexamethasone45 50 29 1113.6S100B, BMP2, CDKN2A, MMP13
32testosterone45 60 11 2413.6CALB2, APOD, MYOG, S100A6, CHGA, TOP2A
33estracyt4510.6PRG2, CHGA
34oligonucleotide4510.6S100A6, PDGFRA, APOD, BMP2, PRG2, CHGA
35nitric oxide45 11 2412.6MMP13, S100A1, BMP2, CDKN2A, PRG2, CHGA
36nicotine45 50 29 1113.6CHGA, BMP2, CDKN2A, NGFR
37genistein45 29 60 2 11 2415.6CDKN2A, PRG2, TOP2A, BMP2, MMP13
38cysteine4510.6S100A6, MYOG, MMP13, NF1, NF2, CHGA
39progesterone45 60 29 11 2414.6KIT, S100A6, CDKN2A, NGFR, NF1, NF2
40tyrosine4510.5CHGA, KIT, MMP13, TOP2A, NF2, PRG2
41calcium45 50 11 2413.5BMP2, PRG2, PDGFRA, S100A6, S100A1, CALB2
42paraffin4510.5NGFR, MYOG, NF1, NF2, CHGA, NES
43retinoic acid45 2411.5S100B, S100A6, S100A1, CDKN2A, KIT, MMP13
44arginine4510.5S100B, PRG2, CHGA, NF1, CDKN2A, BMP2
45nacl4510.5MMP13, NF1, S100A1, S100A6
46deoxyribonucleic acid4510.5BMP2, NF2, CDKN2A
47plp (139-151)6010.4S100B, PRG2
48threonine4510.4S100B, NF2, KIT, CDKN2A, NGFR, BMP2
49nogo-66 (1-40)6010.3PRG2, S100B
50mog (35-55)6010.1S100B, PRG2

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
16Gene Ontology
See all sources

Cellular components related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00557610.3APOD, BMP2, PRG2, CHGA, NGFR, MMP13
2nucleusGO:0056349.9SMARCB1, S100B, S100A6, S100A1, SOX10, PDGFRA

Biological processes related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1negative regulation of cell-matrix adhesionGO:00195310.6CDKN2A, NF1, NF2
2positive regulation of fibroblast proliferationGO:04814610.6S100A6, PDGFRA, NGFR
3positive regulation of apoptotic processGO:04306510.6NGFR, NF1, TOP2A, BMP2, S100B
4negative regulation of cell proliferationGO:00828510.5SMARCB1, CDKN2A, MYOG, NF2, BMP2
5negative regulation of protein kinase activityGO:00646910.5CDKN2A, NF1, NF2
6negative regulation of MAPK cascadeGO:04340910.4NF1, NF2
7positive regulation of transcription from RNA polymerase II promoterGO:04594410.4SOX10, BMP2, TOP2A, MYOG, CDKN2A, SMARCB1
8odontogenesis of dentin-containing toothGO:04247510.4PDGFRA, BMP2, NF2
9positive regulation of phospholipase C activityGO:01086310.3KIT, PDGFRA
10extracellular matrix organizationGO:03019810.1MMP13, NF1, BMP2, PDGFRA

Molecular functions related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein homodimerization activityGO:04280310.3KIT, TOP2A, PDGFRA, S100A1, S100A6, S100B
2S100 protein bindingGO:04454810.3S100A1, S100A6, S100B
3protein bindingGO:0055159.8APOD, PDGFRA, SOX10, S100A1, S100A6, S100B

Products for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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Sources for Malignant Peripheral Nerve Sheath Tumor

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet