MPNST
MCID: MLG077
MIFTS: 57

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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NIH Rare Diseases:42 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 based summary: Malignant Peripheral Nerve Sheath Tumor, also known as MPNST, is related to neurofibromatosis and neuroblastoma. 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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Sources:
48Orphanet, 62UMLS, 8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 57SNOMED-CT, 34MeSH, 39NCIt, 63UMLS via Orphanet
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Malignant Peripheral Nerve Sheath Tumor, Aliases & Descriptions:

Name: Malignant Peripheral Nerve Sheath Tumor 8 42 10 48 62
Mpnst 42 44 48
Malignant Neoplasm of the Peripheral Nerve Sheath 8 62
Malignant Peripheral Nerve Sheath Tumors 44 62
Neurofibrosarcoma 48 62
 
Malignant Neurilemmoma 48
Malignant Neurofibroma 48
Malignant Schwannoma 48
Sarcoma, Neurogenic 62
Neurogenic Sarcoma 48


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
malignant peripheral nerve sheath tumor:
Inheritance: Sporadic; Age of onset: Variable


External Ids:

Disease Ontology8 DOID:5940
MeSH34 D018317
NCIt39 C3798
UMLS via Orphanet63 C0751690

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

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

(show top 50)    (show all 140)
idRelated DiseaseScoreTop Affiliating Genes
1neurofibromatosis32.4NGFR, S100B
2neuroblastoma31.5NGFR
3synovial sarcoma31.5S100B, NES
4neurilemmoma31.3B3GAT1, SMARCB1
5neurofibroma31.2S100B, ACTC1, SOX10, NGFR
6ganglioneuroma31.1NGFR, CHGA, S100B, HMGA1
7sarcoma30.7SMARCB1, NES, ACTC1, S100B, B3GAT1
8gastrointestinal stromal tumor30.7NES, ACTC1, S100B
9melanoma30.5NGFR, SOX10, NES, ACTC1, S100B
10spindle cell sarcoma30.2S100B, ACTC1
11rhabdoid tumor30.2ACTC1, SMARCB1
12rhabdomyosarcoma29.9SMARCB1, NES, ACTC1, SIX1, S100B
13adenocarcinoma29.9HMGA1, ACTC1, CHGA, NES, NGFR
14leiomyosarcoma29.6S100B, ACTC1, NES, SMARCB1
15medulloblastoma29.5SMARCB1, NGFR, NES, ACTC1, S100B
16epithelioid malignant peripheral nerve sheath tumor11.0
17malignant triton tumor10.8
18plexiform neurofibroma10.8
19perineurioma10.7
20neurofibrosarcoma10.7
21cervicitis10.6
22soft tissue sarcoma10.6
23hepatitis10.5
24sinusitis10.5
25malignant melanocytic neoplasm of the peripheral nerve sheath10.5
26thyroiditis10.5
27pheochromocytoma10.5
28malignant perineurioma10.5
29somatostatinoma10.5CHGA
30histiocytosis10.5S100B
31ossifying fibromyxoid tumor10.5B3GAT1
32ganglioglioma10.4CHGA, S100B
33pineocytoma10.4CHGA, S100B
34hodgkin's lymphoma10.3
35hydrocephalus10.3
36monophasic synovial sarcoma10.3
37malignant cardiac peripheral nerve sheath neoplasm10.3
38peritoneal mesothelioma10.3
39meningocele10.3
40melanocytic psammomatous mpnst10.3
41endocervicitis10.3
42nasopharynx carcinoma10.3
43cervix melanoma10.3
44malignant glandular tumor of peripheral nerve sheath10.3
45intussusception10.3
46horner's syndrome10.3
47cellular schwannoma10.3
48cerebritis10.3
49angiosarcoma10.3
50complex regional pain syndrome10.3

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

32
Lung, Bone, Kidney, Breast, Cervix, Brain, Spinal cord, Colon, Liver, Pancreas, Thyroid, Skin, Tongue, Eye, Lymph node, Heart, Smooth muscle, Uterus, Prostate, Testes, Trachea

Animal Models for Malignant Peripheral Nerve Sheath Tumor or affiliated genes

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MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

36
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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Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50)    (show all 448)
idTitleAuthorsYear
1
Malignant peripheral nerve sheath tumor of the parotid gland. (25153067)
2014
2
Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis. (24986157)
2014
3
Genomic and molecular aberrations in malignant peripheral nerve sheath tumor and their roles in personalized target therapy. (23830351)
2013
4
Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle. (23369465)
2013
5
BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. (24366910)
2013
6
Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST. (24426502)
2013
7
Concurrence of malignant peripheral nerve sheath tumor at the site of complex regional pain syndrome type 1 - a case report -. (23614078)
2013
8
Appropriate Modulation of Autophagy Sensitizes Malignant Peripheral Nerve Sheath Tumor Cells to Treatment With Imatinib Mesylate. (24136016)
2013
9
Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. (23084090)
2013
10
Imaging diagnosis-malignant peripheral nerve sheath tumor presenting as an intra-axial brain mass in a young dog. (23363000)
2013
11
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
12
A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion. (22528792)
2012
13
Low-grade malignant triton tumor in the lumbar spine: a rare variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. (21732991)
2012
14
Correspondence regarding "The cytopathology of malignant peripheral nerve sheath tumor: a report of 55 fine-needle aspiration cases". (22714956)
2012
15
Malignant peripheral nerve sheath tumor: the utility of fascicular biopsy and teased fiber studies. (22922579)
2012
16
Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach. (21529197)
2011
17
Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence. (22033963)
2011
18
Orthotopic xenografting of human luciferase-tagged malignant peripheral nerve sheath tumor cells for in vivo testing of candidate therapeutic agents. (21460792)
2011
19
Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. (21540237)
2011
20
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
21
Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1. (20655235)
2010
22
Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five years after diagnosis: a rare finding. (21151396)
2010
23
The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. (20157379)
2010
24
Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. (19521138)
2009
25
Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports. (19830003)
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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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

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Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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

(show all 40)
idCompoundScoreTop Affiliating Genes
1l-amino acid4410.1S100B, CHGA
2catecholamine449.9S100B, CHGA, NGFR
3orcein449.7S100B, ACTC1
4reticulin449.7ACTC1, S100B
5choline44 24 1111.7NGFR, NES, S100B
6bromodeoxyuridine449.7S100B, CHGA, NES
7mucicarmine449.6S100B, ACTC1
8acth449.6B3GAT1, S100B, CHGA
9streptozotocin449.6NES, CHGA
10c-peptide449.6CHGA, NES
11histamine44 28 2411.5B3GAT1, CHGA, NGFR
12s 100449.4S100B, ACTC1, CHGA
13methanol44 2410.4CHGA, ACTC1
14forskolin44 50 1111.4S100B, CHGA, NGFR
15b 723449.4S100B, ACTC1, CHGA
16paclitaxel44 50 1111.4SOX10, CHGA, S100B
17gaba449.4NGFR, NES, CHGA, S100B
18acetylcholine44 50 28 24 1113.4NGFR, NES, CHGA, S100B
19dopamine44 28 24 1112.3NGFR, NES, CHGA, S100B
20formaldehyde44 2410.3S100B, ACTC1, CHGA
21glutamate449.2S100B, CHGA, NES, NGFR
22hematoxylin449.1NGFR, CHGA, ACTC1, S100B
23arginine449.1CHGA, S100B, HMGA1, B3GAT1
24glycogen44 2410.1S100B, ACTC1, CHGA
25fatty acid449.0HMGA1, S100B, CHGA
26dexamethasone44 50 28 1111.9NGFR, CHGA, ACTC1, S100B
27nitric oxide44 24 1110.9B3GAT1, S100B, CHGA, NES, NGFR
28testosterone44 61 24 1111.8CHGA, ACTC1, S100B, B3GAT1
29lipid448.8HMGA1, S100B, CHGA, NES, NGFR
30paraffin448.6NGFR, NES, CHGA, ACTC1, S100B
31steroid448.6HMGA1, S100B, ACTC1, CHGA, NGFR
32vegf448.6NGFR, NES, CHGA, ACTC1, S100B
33oxygen44 249.5NGFR, NES, CHGA, ACTC1, S100B
34matrigel448.4NGFR, NES, CHGA, ACTC1, HMGA1
35progesterone44 28 61 24 1112.1NGFR, NES, CHGA, ACTC1, S100B, B3GAT1
36retinoic acid44 249.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
37glucose448.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
38calcium44 50 24 1111.0NGFR, CHGA, ACTC1, S100B, HMGA1, B3GAT1
39tyrosine448.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
40estrogen447.6B3GAT1, NGFR, NES, CHGA, ACTC1, S100B

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet