MPNST
MCID: MLG077
MIFTS: 56

Malignant Peripheral Nerve Sheath Tumor (MPNST) malady

Rare diseases, Neuronal diseases, Cancer diseases categories

Summaries for Malignant Peripheral Nerve Sheath Tumor

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44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 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:66 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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9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 41NCIt, 59SNOMED-CT, 36MeSH, 60SNOMED-CT via Orphanet, 64UMLS 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):

50
malignant peripheral nerve sheath tumor:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

malignant peripheral nerve sheath tumor 9 44 11 50 63
mpnst 44 46 50
malignant neoplasm of the peripheral nerve sheath 9
malignant peripheral nerve sheath tumors 46
malignant neurofibroma 50
malignant neurilemmoma 50
malignant schwannoma 50
neurogenic sarcoma 50
neurofibrosarcoma 50


External Ids:

Disease Ontology9 DOID:5940
NCIt41 C3798
MeSH36 D018317
SNOMED-CT via Orphanet60 404037002, 19897006, 77418004
UMLS via Orphanet64 C0751690

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

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Sources:
18GeneCards, 19GeneDecks
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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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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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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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34MalaCards
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MalaCards organs/tissues related to Malignant Peripheral Nerve Sheath Tumor:

34
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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38MGI
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MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

38
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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53PubMed
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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, 16Gene 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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46Novoseek, 25HMDB, 12DrugBank, 30IUPHAR, 52PharmGKB, 62Tocris 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 acid4610.1S100B, CHGA
2catecholamine469.9S100B, CHGA, NGFR
3orcein469.7S100B, ACTC1
4reticulin469.7ACTC1, S100B
5choline46 25 1211.7NGFR, NES, S100B
6bromodeoxyuridine469.7S100B, CHGA, NES
7mucicarmine469.6S100B, ACTC1
8acth469.6B3GAT1, S100B, CHGA
9streptozotocin469.6NES, CHGA
10c-peptide469.6CHGA, NES
11histamine46 30 2511.5B3GAT1, CHGA, NGFR
12s 100469.4S100B, ACTC1, CHGA
13methanol46 2510.4CHGA, ACTC1
14forskolin46 52 1211.4S100B, CHGA, NGFR
15b 723469.4S100B, ACTC1, CHGA
16paclitaxel46 52 1211.4SOX10, CHGA, S100B
17gaba469.4NGFR, NES, CHGA, S100B
18acetylcholine46 52 30 25 1213.4NGFR, NES, CHGA, S100B
19dopamine46 30 25 1212.3NGFR, NES, CHGA, S100B
20formaldehyde46 2510.3S100B, ACTC1, CHGA
21glutamate469.2S100B, CHGA, NES, NGFR
22hematoxylin469.1NGFR, CHGA, ACTC1, S100B
23arginine469.1CHGA, S100B, HMGA1, B3GAT1
24glycogen46 2510.1S100B, ACTC1, CHGA
25fatty acid469.0HMGA1, S100B, CHGA
26dexamethasone46 52 30 1211.9NGFR, CHGA, ACTC1, S100B
27nitric oxide46 25 1210.9B3GAT1, S100B, CHGA, NES, NGFR
28testosterone46 62 25 1211.8CHGA, ACTC1, S100B, B3GAT1
29lipid468.8HMGA1, S100B, CHGA, NES, NGFR
30paraffin468.6NGFR, NES, CHGA, ACTC1, S100B
31steroid468.6HMGA1, S100B, ACTC1, CHGA, NGFR
32vegf468.6NGFR, NES, CHGA, ACTC1, S100B
33oxygen46 259.5NGFR, NES, CHGA, ACTC1, S100B
34matrigel468.4NGFR, NES, CHGA, ACTC1, HMGA1
35progesterone46 30 62 25 1212.1NGFR, NES, CHGA, ACTC1, S100B, B3GAT1
36retinoic acid46 259.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
37glucose468.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
38calcium46 52 25 1211.0NGFR, CHGA, ACTC1, S100B, HMGA1, B3GAT1
39tyrosine468.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
40estrogen467.6B3GAT1, NGFR, NES, CHGA, ACTC1, S100B

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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17Gene 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
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet