MCID: MLG077
MIFTS: 48

Malignant Peripheral Nerve Sheath Tumor malady

Rare diseases, Neuronal diseases, Cancer diseases categories

Summaries for Malignant Peripheral Nerve Sheath Tumor

About this section


NIH Rare Diseases:41 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 malignant neurilemmoma, 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:63 A malignant peripheral nerve sheath tumor (MPNST) (also known as \"Malignant schwannoma,\"... more...

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

About this section
Sources:
41NIH Rare Diseases, 47Orphanet, 60UMLS, 9Disease Ontology, 11DISEASES, 43Novoseek, 55SNOMED-CT, 38NCIt, 33MeSH, 61UMLS via Orphanet
See all sources

Malignant Peripheral Nerve Sheath Tumor, Aliases & Descriptions:

Name: Malignant Peripheral Nerve Sheath Tumor 9 41 11 47 60
Malignant Neurilemmoma 41 43 47
Neurofibrosarcoma 41 47 60
Mpnst 41 43 47
Malignant Neurofibroma 41 47
 
Malignant Schwannoma 41 47
Neurogenic Sarcoma 41 47
Malignant Neoplasm of the Peripheral Nerve Sheath 9
Malignant Peripheral Nerve Sheath Tumors 43


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
malignant peripheral nerve sheath tumor:
Inheritance: Not applicable; Prevalence: 1-9/100000 (Worldwide); Age of onset: Adolescent,Adult,Childhood,Elderly,Infancy; Age of death: adolescent,adult,early childhood,elderly,infantile,late childhood,young Adult


External Ids:

Disease Ontology9 DOID:5940
NCIt38 C3798
MeSH33 D018317
Orphanet47 3148
UMLS via Orphanet61 C0751690

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

About this section

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

(show top 50)    (show all 139)
idRelated DiseaseScoreTop Affiliating Genes
1neurofibromatosis32.3NGFR, S100B
2neuroblastoma31.5NGFR
3neurilemmoma31.3B3GAT1, SMARCB1
4neurofibroma31.2S100B, ACTC1, SOX10, NGFR
5ganglioneuroma31.1NGFR, CHGA, S100B, HMGA1
6ewing sarcoma30.7NES, CHGA, S100B
7sarcoma30.7SMARCB1, NES, ACTC1, S100B, B3GAT1
8gastrointestinal stromal tumor30.7NES, ACTC1, S100B
9melanoma30.5NGFR, SOX10, NES, ACTC1, S100B
10spindle cell sarcoma30.2S100B, ACTC1
11rhabdoid tumor30.1ACTC1, SMARCB1
12rhabdomyosarcoma29.9SMARCB1, NES, ACTC1, SIX1, S100B
13adenocarcinoma29.8HMGA1, ACTC1, CHGA, NES, NGFR
14leiomyosarcoma29.6S100B, ACTC1, NES, SMARCB1
15medulloblastoma29.4SMARCB1, NGFR, NES, ACTC1, S100B
16neurofibromatosis, type 111.1
17epithelioid malignant peripheral nerve sheath tumor11.0
18malignant triton tumor10.8
19plexiform neurofibroma10.8
20perineurioma10.7
21neurofibrosarcoma10.7
22cervicitis10.7
23soft tissue sarcoma10.6
24pheochromocytoma10.5
25melanoma and neural system tumor syndrome10.5
26hepatitis10.5
27sinusitis10.5
28angiosarcoma10.5
29thyroiditis10.5
30malignant perineurioma10.5
31mesothelioma10.5
32somatostatinoma10.5CHGA
33histiocytosis10.5S100B
34ossifying fibromyxoid tumor10.5B3GAT1
35ganglioglioma10.4CHGA, S100B
36pineocytoma10.4CHGA, S100B
37lung cancer10.3
38wilms tumor10.3
39schwannomatosis10.3
40neurofibromatosis, type 210.3
41hydrocephalus10.3
42peritoneal mesothelioma10.3
43meningocele10.3
44nasopharynx carcinoma10.3
45monophasic synovial sarcoma10.3
46malignant glandular tumor of peripheral nerve sheath10.3
47intussusception10.3
48melanocytic psammomatous mpnst10.3
49horner's syndrome10.3
50cellular schwannoma10.3

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

About this section

Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

About this section

Drug clinical trials:

Search ClinicalTrials for Malignant Peripheral Nerve Sheath Tumor

Search NIH Clinical Center for Malignant Peripheral Nerve Sheath Tumor

Genetic Tests for Malignant Peripheral Nerve Sheath Tumor

About this section

Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

About this section

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

31
Lung, Bone, Kidney, Breast, Cervix, Brain, Lymph node, Spinal cord, Colon, Liver, Pancreas, Thyroid, Skin, Tongue, Eye, Heart, Smooth muscle, Uterus, Prostate, Testes, Trachea

Animal Models for Malignant Peripheral Nerve Sheath Tumor or affiliated genes

About this section

MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

35
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

About this section

Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50)    (show all 467)
idTitleAuthorsYear
1
Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. (25443687)
2015
2
Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors. (25598015)
2015
3
Malignant peripheral nerve sheath tumor of the parotid gland. (25153067)
2014
4
Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis. (24986157)
2014
5
Genomic and molecular aberrations in malignant peripheral nerve sheath tumor and their roles in personalized target therapy. (23830351)
2013
6
Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle. (23369465)
2013
7
BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. (24366910)
2013
8
Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST. (24426502)
2013
9
Concurrence of malignant peripheral nerve sheath tumor at the site of complex regional pain syndrome type 1 - a case report -. (23614078)
2013
10
Appropriate Modulation of Autophagy Sensitizes Malignant Peripheral Nerve Sheath Tumor Cells to Treatment With Imatinib Mesylate. (24136016)
2013
11
Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. (23084090)
2013
12
Imaging diagnosis-malignant peripheral nerve sheath tumor presenting as an intra-axial brain mass in a young dog. (23363000)
2013
13
A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion. (22528792)
2012
14
Low-grade malignant triton tumor in the lumbar spine: a rare variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. (21732991)
2012
15
Correspondence regarding "The cytopathology of malignant peripheral nerve sheath tumor: a report of 55 fine-needle aspiration cases". (22714956)
2012
16
Malignant peripheral nerve sheath tumor: the utility of fascicular biopsy and teased fiber studies. (22922579)
2012
17
Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach. (21529197)
2011
18
Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence. (22033963)
2011
19
Orthotopic xenografting of human luciferase-tagged malignant peripheral nerve sheath tumor cells for in vivo testing of candidate therapeutic agents. (21460792)
2011
20
Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. (21540237)
2011
21
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
22
Prognostic significance of p14ARF, p15INK4b, and p16INK4a inactivation in malignant peripheral nerve sheath tumors. (21262917)
2011
23
Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1. (20655235)
2010
24
Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five years after diagnosis: a rare finding. (21151396)
2010
25
The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. (20157379)
2010
26
Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. (19521138)
2009
27
Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports. (19830003)
2009
28
Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. (18460849)
2008
29
Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). (17960768)
2008
30
EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy. (18650488)
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

About this section

Expression for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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

Pathways for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

About this section

Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

About this section

Compounds related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

(show all 40)
idCompoundScoreTop Affiliating Genes
1l-amino acid4310.1S100B, CHGA
2catecholamine439.9S100B, CHGA, NGFR
3orcein439.7S100B, ACTC1
4reticulin439.7ACTC1, S100B
5choline43 24 1211.7NGFR, NES, S100B
6bromodeoxyuridine439.7S100B, CHGA, NES
7mucicarmine439.6S100B, ACTC1
8acth439.6B3GAT1, S100B, CHGA
9streptozotocin439.6NES, CHGA
10c-peptide439.6CHGA, NES
11histamine43 28 2411.5B3GAT1, CHGA, NGFR
12s 100439.4S100B, ACTC1, CHGA
13methanol43 2410.4CHGA, ACTC1
14forskolin43 49 1211.4S100B, CHGA, NGFR
15b 723439.4S100B, ACTC1, CHGA
16paclitaxel43 49 1211.4SOX10, CHGA, S100B
17gaba439.4NGFR, NES, CHGA, S100B
18acetylcholine43 49 28 24 1213.4NGFR, NES, CHGA, S100B
19dopamine43 28 24 1212.3NGFR, NES, CHGA, S100B
20formaldehyde43 2410.3S100B, ACTC1, CHGA
21glutamate439.2S100B, CHGA, NES, NGFR
22hematoxylin439.1NGFR, CHGA, ACTC1, S100B
23arginine439.1CHGA, S100B, HMGA1, B3GAT1
24glycogen43 2410.1S100B, ACTC1, CHGA
25fatty acid439.0HMGA1, S100B, CHGA
26dexamethasone43 49 28 1211.9NGFR, CHGA, ACTC1, S100B
27nitric oxide43 24 1210.9B3GAT1, S100B, CHGA, NES, NGFR
28testosterone43 59 24 1211.8CHGA, ACTC1, S100B, B3GAT1
29lipid438.8HMGA1, S100B, CHGA, NES, NGFR
30paraffin438.6NGFR, NES, CHGA, ACTC1, S100B
31steroid438.6HMGA1, S100B, ACTC1, CHGA, NGFR
32vegf438.6NGFR, NES, CHGA, ACTC1, S100B
33oxygen43 249.5NGFR, NES, CHGA, ACTC1, S100B
34matrigel438.4NGFR, NES, CHGA, ACTC1, HMGA1
35progesterone43 28 59 24 1212.1NGFR, NES, CHGA, ACTC1, S100B, B3GAT1
36retinoic acid43 249.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
37glucose438.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
38calcium43 49 24 1211.0NGFR, CHGA, ACTC1, S100B, HMGA1, B3GAT1
39tyrosine438.0NGFR, NES, CHGA, ACTC1, S100B, HMGA1
40estrogen437.6B3GAT1, NGFR, NES, CHGA, ACTC1, S100B

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

About this section

Biological processes related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1nucleosome disassemblyGO:00063379.6HMGA1, SMARCB1
2central nervous system developmentGO:00074179.0S100B, NES, NGFR

Products for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Malignant Peripheral Nerve Sheath Tumor

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet