MPNST
MCID: MLG077
MIFTS: 56

Malignant Peripheral Nerve Sheath Tumor (MPNST) malady

Rare diseases, Neuronal diseases, Cancer diseases categories
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Summaries for Malignant Peripheral Nerve Sheath Tumor

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43NIH Rare Diseases, 65Wikipedia, 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 neurofibroma. An important gene associated with Malignant Peripheral Nerve Sheath Tumor is S100B (S100 calcium binding protein B). The compounds l-amino acid and catecholamine have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and kidney, and related mouse phenotypes are digestive/alimentary and behavior/neurological.

Wikipedia:65 A malignant peripheral nerve sheath tumor (MPNST) (also known as \"Malignant schwannoma,\"... more...

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

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8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 40NCIt, 58SNOMED-CT, 35MeSH, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

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


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 62
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
NCIt40 C3798
MeSH35 D018317
SNOMED-CT via Orphanet59 404037002, 19897006, 77418004
UMLS via Orphanet63 C0751690

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

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17GeneCards, 18GeneDecks
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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 139)
idRelated DiseaseScoreTop Affiliating Genes
1neurofibromatosis32.0NGFR, S100B
2neurofibroma31.4S100B, ACTC1, SOX10, NGFR
3synovial sarcoma31.2S100B, NES
4sarcoma31.1SMARCB1, NES, ACTC1, S100B, B3GAT1
5ganglioneuroma31.1NGFR, CHGA, S100B, HMGA1
6melanoma31.0NGFR, SOX10, NES, ACTC1, S100B
7neurilemmoma30.9B3GAT1, SMARCB1
8neuroblastoma30.9NGFR
9gastrointestinal stromal tumor30.7NES, ACTC1, S100B
10rhabdomyosarcoma30.4SMARCB1, NES, ACTC1, SIX1, S100B
11adenocarcinoma30.3HMGA1, ACTC1, CHGA, NES, NGFR
12rhabdoid tumor30.0ACTC1, SMARCB1
13spindle cell sarcoma30.0S100B, ACTC1
14leiomyosarcoma29.9S100B, ACTC1, NES, SMARCB1
15medulloblastoma29.8SMARCB1, NGFR, NES, ACTC1, S100B
16epithelioid malignant peripheral nerve sheath tumor11.0
17malignant triton tumor10.8
18plexiform neurofibroma10.8
19neurofibrosarcoma10.6
20perineurioma10.6
21cervicitis10.6
22angiosarcoma10.5
23hepatitis10.5
24thyroiditis10.5
25pheochromocytoma10.5
26soft tissue sarcoma10.5
27malignant perineurioma10.5
28meningocele10.3
29peritoneal mesothelioma10.3
30monophasic synovial sarcoma10.3
31malignant glandular tumor of peripheral nerve sheath10.3
32intussusception10.3
33melanocytic psammomatous mpnst10.3
34horner's syndrome10.3
35cellular schwannoma10.3
36cervix melanoma10.3
37cerebritis10.3
38endocervicitis10.3
39malignant cardiac peripheral nerve sheath neoplasm10.3
40complex regional pain syndrome10.3
41dermatomyositis10.3
42hodgkin's lymphoma10.3
43liposarcoma10.3
44lung cancer10.3
45malignant melanocytic neoplasm of the peripheral nerve sheath10.3
46neuroma10.3
47neuropathy10.3
48papillary thyroid carcinoma10.3
49peritonitis10.3
50prostatitis10.3

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



Diseases related to malignant peripheral nerve sheath tumor

Symptoms for Malignant Peripheral Nerve Sheath Tumor

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Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

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Genetic Tests for Malignant Peripheral Nerve Sheath Tumor

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Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

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33MalaCards
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MalaCards organs/tissues related to Malignant Peripheral Nerve Sheath Tumor:

33
Lung, Bone, Kidney, Breast, Cervix, Brain, Thyroid, Pancreas, Liver, Colon, Spinal cord, Tongue, Skin, Prostate, Testes, Uterus, Eye, Smooth muscle, Heart, Lymph node, Trachea

Animal Models for Malignant Peripheral Nerve Sheath Tumor or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.7SIX1, SOX10, NGFR, SMARCB1
2MP:00053867.9NGFR, SOX10, NES, SIX1, S100B, B3GAT1
3MP:00053857.8SMARCB1, NGFR, CHGA, ACTC1, SIX1, HMGA1
4MP:00036317.7SMARCB1, NGFR, SOX10, NES, SIX1, B3GAT1
5MP:00053847.4SMARCB1, NGFR, SOX10, NES, ACTC1, SIX1
6MP:00107687.2SMARCB1, NGFR, SOX10, NES, CHGA, ACTC1
7MP:00053767.2SMARCB1, NGFR, SOX10, CHGA, ACTC1, HMGA1
8MP:00053786.7HMGA1, SMARCB1, NGFR, SOX10, NES, CHGA

Publications for Malignant Peripheral Nerve Sheath Tumor

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52PubMed
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Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50)    (show all 424)
idTitleAuthorsYear
1
Genomic and molecular aberrations in malignant peripheral nerve sheath tumor and their roles in personalized target therapy. (23830351)
2013
2
Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle. (23369465)
2013
3
BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. (24366910)
2013
4
Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST. (24426502)
2013
5
Concurrence of malignant peripheral nerve sheath tumor at the site of complex regional pain syndrome type 1 - a case report -. (23614078)
2013
6
Appropriate Modulation of Autophagy Sensitizes Malignant Peripheral Nerve Sheath Tumor Cells to Treatment With Imatinib Mesylate. (24136016)
2013
7
Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. (23084090)
2013
8
Imaging diagnosis-malignant peripheral nerve sheath tumor presenting as an intra-axial brain mass in a young dog. (23363000)
2013
9
Sensitivity of malignant peripheral nerve sheath tumor cells to TRAIL is augmented by loss of NF1 through modulation of MYC/MAD and is potentiated by curcumin through induction of ROS. (23437333)
2013
10
A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion. (22528792)
2012
11
Low-grade malignant triton tumor in the lumbar spine: a rare variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. (21732991)
2012
12
Correspondence regarding "The cytopathology of malignant peripheral nerve sheath tumor: a report of 55 fine-needle aspiration cases". (22714956)
2012
13
Malignant peripheral nerve sheath tumor: the utility of fascicular biopsy and teased fiber studies. (22922579)
2012
14
Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach. (21529197)
2011
15
Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence. (22033963)
2011
16
Orthotopic xenografting of human luciferase-tagged malignant peripheral nerve sheath tumor cells for in vivo testing of candidate therapeutic agents. (21460792)
2011
17
Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. (21540237)
2011
18
False-negative fluorine-18 fluorodeoxyglucose positron emission tomography of a malignant peripheral nerve sheath tumor arising from a plexiform neurofibroma in the setting of neurofibromatosis type 1. (21838076)
2011
19
Prognostic significance of p14ARF, p15INK4b, and p16INK4a inactivation in malignant peripheral nerve sheath tumors. (21262917)
2011
20
Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1. (20655235)
2010
21
Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five years after diagnosis: a rare finding. (21151396)
2010
22
The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. (20157379)
2010
23
Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. (19521138)
2009
24
Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports. (19830003)
2009
25
Neuregulin-1 beta and neuregulin-1 alpha differentially affect the migration and invasion of malignant peripheral nerve sheath tumor cells. (19306381)
2009
26
Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. (18460849)
2008
27
Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). (17960768)
2008
28
EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy. (18650488)
2008
29
Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report. (18753958)
2008
30
Fine-needle aspiration biopsy of metastatic malignant melanoma resembling a malignant peripheral nerve sheath tumor. (18773432)
2008
31
Malignant peripheral nerve sheath tumor in a hamster. (17994674)
2007
32
Brain metastases as primary manifestation of a melanocytic malignant peripheral nerve sheath tumor in a 60-year-old man. (17227576)
2007
33
Nestin expression as a new marker in malignant peripheral nerve sheath tumors. (17300669)
2007
34
Management of childhood malignant peripheral nerve sheath tumor. (17705563)
2007
35
Multiple intracranial metastases from a malignant peripheral nerve sheath tumor of the extremities. (16598432)
2006
36
Malignant peripheral nerve sheath tumor of the kidney. (16448437)
2006
37
Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy. (16182351)
2006
38
Primary malignant peripheral nerve-sheath tumor of the common bile duct. (15830292)
2005
39
Molecular profiling of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1, based on large-scale real-time RT-PCR. (15255999)
2004
40
Malignant peripheral nerve sheath tumor of the vulva. A case report. (12380453)
2002
41
Malignant peripheral nerve sheath tumor of the cauda equina. (11731882)
2001
42
Fine-needle aspiration cytology and core biopsy of malignant peripheral nerve sheath tumor of the uterus: a case report. (11335967)
2001
43
Malignant peripheral nerve sheath tumor of the esophagus. (11155010)
2000
44
Malignant peripheral nerve sheath tumor arising in benign ancient schwannoma: a case report with an immunohistochemical study. (10792776)
2000
45
Usefulness of cytokeratin subsets for distinguishing monophasic synovial sarcoma from malignant peripheral nerve sheath tumor. (10549251)
1999
46
Malignant peripheral nerve sheath tumor in a cat. (10608693)
1999
47
Malignant peripheral nerve sheath tumor in a water moccasin (Agkistrodon piscivorus). (9580548)
1998
48
Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. (9770186)
1998
49
Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. (9808129)
1998
50
Malignant peripheral nerve sheath tumor: a fine-needle aspiration biopsy study. (9316788)
1997

Variations for Malignant Peripheral Nerve Sheath Tumor

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Expression for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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Pathways for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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45Novoseek, 24HMDB, 11DrugBank, 29IUPHAR, 51PharmGKB, 61Tocris Bioscience
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Compounds related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

(show all 40)
idCompoundScoreTop Affiliating Genes
1l-amino acid4510.1S100B, CHGA
2catecholamine459.9S100B, CHGA, NGFR
3orcein459.7S100B, ACTC1
4reticulin459.7ACTC1, S100B
5choline45 24 1111.7NGFR, NES, S100B
6bromodeoxyuridine459.7S100B, CHGA, NES
7mucicarmine459.6S100B, ACTC1
8acth459.6B3GAT1, S100B, CHGA
9streptozotocin459.6NES, CHGA
10c-peptide459.6CHGA, NES
11histamine45 29 2411.5B3GAT1, CHGA, NGFR
12s 100459.4S100B, ACTC1, CHGA
13methanol45 2410.4CHGA, ACTC1
14forskolin45 51 1111.4S100B, CHGA, NGFR
15b 723459.4S100B, ACTC1, CHGA
16paclitaxel45 51 1111.4SOX10, CHGA, S100B
17gaba459.4NGFR, NES, CHGA, S100B
18acetylcholine45 51 29 24 1113.4NGFR, NES, CHGA, S100B
19dopamine45 29 24 1112.3NGFR, NES, CHGA, S100B
20formaldehyde45 2410.3S100B, ACTC1, CHGA
21glutamate459.2S100B, CHGA, NES, NGFR
22hematoxylin459.1NGFR, CHGA, ACTC1, S100B
23arginine459.1CHGA, S100B, HMGA1, B3GAT1
24glycogen45 2410.1S100B, ACTC1, CHGA
25fatty acid459.0HMGA1, S100B, CHGA
26dexamethasone45 51 29 1111.9NGFR, CHGA, ACTC1, S100B
27nitric oxide45 24 1110.9B3GAT1, S100B, CHGA, NES, NGFR
28testosterone45 61 24 1111.8CHGA, ACTC1, S100B, B3GAT1
29lipid458.8HMGA1, S100B, CHGA, NES, NGFR
30paraffin458.6NGFR, NES, CHGA, ACTC1, S100B
31steroid458.6HMGA1, S100B, ACTC1, CHGA, NGFR
32vegf458.6NGFR, NES, CHGA, ACTC1, S100B
33oxygen45 249.5NGFR, NES, CHGA, ACTC1, S100B
34matrigel458.4NGFR, NES, CHGA, ACTC1, HMGA1
35progesterone45 29 61 24 1112.1NGFR, NES, CHGA, ACTC1, S100B, B3GAT1
36retinoic acid45 249.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
37glucose458.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
38calcium45 51 24 1111.0NGFR, CHGA, ACTC1, S100B, HMGA1, B3GAT1
39tyrosine458.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
40estrogen457.6B3GAT1, NGFR, NES, CHGA, ACTC1, S100B

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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16Gene Ontology
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Biological processes related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1nucleosome disassemblyGO:0063379.6HMGA1, SMARCB1
2central nervous system developmentGO:0074179.0S100B, NES, NGFR

Products for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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Sources for Malignant Peripheral Nerve Sheath Tumor

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet