Malignant Peripheral Nerve Sheath Tumor disease: Malacards - Research Articles, Symptoms, Drugs, Genes, Clinical Trials

Summaries for Malignant Peripheral Nerve Sheath Tumor

Sources:
⁴²NIH Rare Diseases, ⁶⁴Wikipedia, ³²MalaCards
See all sources

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

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

Sources:
⁴⁸Orphanet, ⁶¹UMLS, ⁹Disease Ontology, ⁴²NIH Rare Diseases, ¹¹DISEASES, ⁴⁴Novoseek, ⁵⁶SNOMED-CT, ³⁴MeSH, ³⁹NCIt, ⁶²UMLS via Orphanet
See all sources

Malignant Peripheral Nerve Sheath Tumor, Aliases & Descriptions:

Name: Malignant Peripheral Nerve Sheath Tumor ⁹ ⁴² ¹¹ ⁴⁸ ⁶¹

Mpnst ⁴² ⁴⁴ ⁴⁸

Malignant Neoplasm of the Peripheral Nerve Sheath ⁹ ⁶¹

Malignant Peripheral Nerve Sheath Tumors ⁴⁴ ⁶¹

Neurofibrosarcoma ⁴⁸ ⁶¹

Malignant Neurilemmoma ⁴⁸

Malignant Neurofibroma ⁴⁸

Malignant Schwannoma ⁴⁸

Sarcoma, Neurogenic ⁶¹

Neurogenic Sarcoma ⁴⁸

Classifications:

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

Orphanet: ⁴⁸

Rare neurological diseases

Rare tumors

Characteristics (Orphanet epidemiological data):

⁴⁸

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

External Ids:

Disease Ontology⁹ DOID:5940

MeSH³⁴ D018317

SNOMED-CT⁵⁶ 19897006, 134324009, 404037002

NCIt³⁹ C3798

UMLS via Orphanet⁶² C0751690

UMLS⁶¹ C0751690

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

Sources:
¹⁸GeneCards, ¹⁹GeneDecks
See all sources

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

(show top 50) (show all 140)

id	Related Disease	Score	Top Affiliating Genes
1	neurofibromatosis	32.4	NGFR, S100B
2	neuroblastoma	31.5	NGFR
3	synovial sarcoma	31.5	S100B, NES
4	neurilemmoma	31.3	B3GAT1, SMARCB1
5	neurofibroma	31.2	S100B, ACTC1, SOX10, NGFR
6	ganglioneuroma	31.1	NGFR, CHGA, S100B, HMGA1
7	sarcoma	30.7	SMARCB1, NES, ACTC1, S100B, B3GAT1
8	gastrointestinal stromal tumor	30.7	NES, ACTC1, S100B
9	melanoma	30.5	NGFR, SOX10, NES, ACTC1, S100B
10	spindle cell sarcoma	30.2	S100B, ACTC1
11	rhabdoid tumor	30.2	ACTC1, SMARCB1
12	rhabdomyosarcoma	29.9	SMARCB1, NES, ACTC1, SIX1, S100B
13	adenocarcinoma	29.9	HMGA1, ACTC1, CHGA, NES, NGFR
14	leiomyosarcoma	29.6	S100B, ACTC1, NES, SMARCB1
15	medulloblastoma	29.5	SMARCB1, NGFR, NES, ACTC1, S100B
16	epithelioid malignant peripheral nerve sheath tumor	11.0
17	malignant triton tumor	10.8
18	plexiform neurofibroma	10.8
19	perineurioma	10.7
20	neurofibrosarcoma	10.7
21	cervicitis	10.6
22	soft tissue sarcoma	10.6
23	hepatitis	10.5
24	sinusitis	10.5
25	malignant melanocytic neoplasm of the peripheral nerve sheath	10.5
26	thyroiditis	10.5
27	pheochromocytoma	10.5
28	malignant perineurioma	10.5
29	somatostatinoma	10.5	CHGA
30	histiocytosis	10.5	S100B
31	ossifying fibromyxoid tumor	10.5	B3GAT1
32	ganglioglioma	10.4	CHGA, S100B
33	pineocytoma	10.4	CHGA, S100B
34	hodgkin's lymphoma	10.3
35	hydrocephalus	10.3
36	monophasic synovial sarcoma	10.3
37	malignant cardiac peripheral nerve sheath neoplasm	10.3
38	peritoneal mesothelioma	10.3
39	meningocele	10.3
40	melanocytic psammomatous mpnst	10.3
41	endocervicitis	10.3
42	nasopharynx carcinoma	10.3
43	cervix melanoma	10.3
44	malignant glandular tumor of peripheral nerve sheath	10.3
45	intussusception	10.3
46	horner's syndrome	10.3
47	cellular schwannoma	10.3
48	cerebritis	10.3
49	angiosarcoma	10.3
50	complex regional pain syndrome	10.3

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

Symptoms for Malignant Peripheral Nerve Sheath Tumor

Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

Sources:
⁴¹NIH Clinical Center, ⁶ClinicalTrials, ⁶¹UMLS, ⁴⁰NDF-RT
See all sources

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

Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

Sources:
³²MalaCards
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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

Sources:
³⁶MGI
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MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

³⁶

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	digestive/alimentary phenotype	MP:0005381	8.7	SIX1, SOX10, NGFR, SMARCB1
2	behavior/neurological phenotype	MP:0005386	7.9	NGFR, SOX10, NES, SIX1, S100B, B3GAT1
3	cardiovascular system phenotype	MP:0005385	7.8	SMARCB1, NGFR, CHGA, ACTC1, SIX1, HMGA1
4	nervous system phenotype	MP:0003631	7.7	SMARCB1, NGFR, SOX10, NES, SIX1, B3GAT1
5	cellular phenotype	MP:0005384	7.4	SMARCB1, NGFR, SOX10, NES, ACTC1, SIX1
6	mortality/aging	MP:0010768	7.2	SMARCB1, NGFR, SOX10, NES, CHGA, ACTC1
7	homeostasis/metabolism phenotype	MP:0005376	7.2	SMARCB1, NGFR, SOX10, CHGA, ACTC1, HMGA1
8	growth/size/body phenotype	MP:0005378	6.7	HMGA1, SMARCB1, NGFR, SOX10, NES, CHGA

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 448)

id	Title	Authors	Year
1	Malignant peripheral nerve sheath tumor of the parotid gland. (25153067)	Chis O.... Albu S.	2014
2	Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis. (24986157)	Thomas J.G.... Gopinath S.P.	2014
3	Genomic and molecular aberrations in malignant peripheral nerve sheath tumor and their roles in personalized target therapy. (23830351)	Yang J.... Du X.	2013
4	Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle. (23369465)	Pavarini S.P.... Farias da Cruz C.E.	2013
5	BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. (24366910)	Hirbe A.C.... Gutmann D.H.	2013
6	Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST. (24426502)	Leena J.B.... Swethadri G.K.	2013
7	Concurrence of malignant peripheral nerve sheath tumor at the site of complex regional pain syndrome type 1 - a case report -. (23614078)	Jeong Y.H.... Nahm F.S.	2013
8	Appropriate Modulation of Autophagy Sensitizes Malignant Peripheral Nerve Sheath Tumor Cells to Treatment With Imatinib Mesylate. (24136016)	Okano M.... Takemura T.	2013
9	Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. (23084090)	Kamran S.C.... Ramaiya N.H.	2013
10	Imaging diagnosis-malignant peripheral nerve sheath tumor presenting as an intra-axial brain mass in a young dog. (23363000)	Shihab N.... Volk H.A.	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)	Reuss D.E.... von Deimling A.	2013
12	A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion. (22528792)	Yan B.... Wei P.	2012
13	Low-grade malignant triton tumor in the lumbar spine: a rare variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. (21732991)	Prieto R.... GonzA!lez-Llanos F.	2012
14	Correspondence regarding "The cytopathology of malignant peripheral nerve sheath tumor: a report of 55 fine-needle aspiration cases". (22714956)	JimAcnez-Heffernan J.A.... Viguer J.M.	2012
15	Malignant peripheral nerve sheath tumor: the utility of fascicular biopsy and teased fiber studies. (22922579)	Tracy J.A.... Dyck P.J.	2012
16	Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach. (21529197)	McLaughlin E.J.... Storm P.B.	2011
17	Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence. (22033963)	Edizer D.T.... IA9A+ldak H.	2011
18	Orthotopic xenografting of human luciferase-tagged malignant peripheral nerve sheath tumor cells for in vivo testing of candidate therapeutic agents. (21460792)	Turk A.N.... Carroll S.L.	2011
19	Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. (21540237)	Torres K.E.... Lev D.	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)	Shahid K.R.... Spinner R.J.	2011
21	Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1. (20655235)	Ge P.... Luo Y.	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)	Godfrey G.J.... Farghaly H.	2010
23	The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. (20157379)	Lee C.S.... Park J.K.	2010
24	Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. (19521138)	Chamoun R.B.... Jea A.	2009
25	Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports. (19830003)	Kosmas C.... Karabelis A.	2009
26	Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. (18460849)	Niwa T.... Inoue T.	2008
27	Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). (17960768)	Upadhyaya M.... Mautner V.	2008
28	EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy. (18650488)	Holtkamp N.... von Deimling A.	2008
29	Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report. (18753958)	Pusceddu S.... Ditto A.	2008
30	Fine-needle aspiration biopsy of metastatic malignant melanoma resembling a malignant peripheral nerve sheath tumor. (18773432)	Bardarov S.... Pu R.T.	2008
31	Malignant peripheral nerve sheath tumor in a hamster. (17994674)	Snyder L.A.... Neel J.A.	2007
32	Brain metastases as primary manifestation of a melanocytic malignant peripheral nerve sheath tumor in a 60-year-old man. (17227576)	Tilgner J.... Vesper J.	2007
33	Nestin expression as a new marker in malignant peripheral nerve sheath tumors. (17300669)	Shimada S.... Illei P.B.	2007
34	Management of childhood malignant peripheral nerve sheath tumor. (17705563)	Ferrari A.... Carli M.	2007
35	Multiple intracranial metastases from a malignant peripheral nerve sheath tumor of the extremities. (16598432)	Stark A.M.... Mehdorn H.M.	2006
36	Malignant peripheral nerve sheath tumor of the kidney. (16448437)	Williams S.B.... Manyak M.J.	2006
37	Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy. (16182351)	Rodriguez A.O.... Leiserowitz G.S.	2006
38	Primary malignant peripheral nerve-sheath tumor of the common bile duct. (15830292)	Matsuo K.... Shimada H.	2005
39	Molecular profiling of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1, based on large-scale real-time RT-PCR. (15255999)	LAcvy P.... BiA"che I.	2004
40	Malignant peripheral nerve sheath tumor of the vulva. A case report. (12380453)	Maglione M.A.... Calandria L.	2002
41	Malignant peripheral nerve sheath tumor of the cauda equina. (11731882)	Acharya R.... Sehgal A.D.	2001
42	Fine-needle aspiration cytology and core biopsy of malignant peripheral nerve sheath tumor of the uterus: a case report. (11335967)	Molina C.P.... LogroA+o R.	2001
43	Malignant peripheral nerve sheath tumor of the esophagus. (11155010)	Manger T.... Lippert H.	2000
44	Malignant peripheral nerve sheath tumor arising in benign ancient schwannoma: a case report with an immunohistochemical study. (10792776)	Mikami Y.... Manabe T.	2000
45	Usefulness of cytokeratin subsets for distinguishing monophasic synovial sarcoma from malignant peripheral nerve sheath tumor. (10549251)	Smith T.A.... Goldblum J.R.	1999
46	Malignant peripheral nerve sheath tumor in a cat. (10608693)	Watrous B.J.... Normal L.M.	1999
47	Malignant peripheral nerve sheath tumor in a water moccasin (Agkistrodon piscivorus). (9580548)	Ramis A.... Zidan A.	1998
48	Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. (9770186)	Khan R.J.... Chughtai A.S.	1998
49	Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. (9808129)	Hirose T.... Sano T.	1998
50	Malignant peripheral nerve sheath tumor: a fine-needle aspiration biopsy study. (9316788)	McGee R.S.... Kilpatrick S.E.	1997

Genes related to Malignant Peripheral Nerve Sheath Tumor

Sources:
¹⁸GeneCards, ¹antibodies-online, ⁴⁵Novus Biologicals
See all sources

Genes related to Malignant Peripheral Nerve Sheath Tumor:

- Elite gene

id	Symbol	Description	Score	Implication
1	S100B	S100 calcium binding protein B	21.14	GeneCards inferred via : Publications, Disorders (show sections)
2	NGFR	nerve growth factor receptor	19.14	GeneCards inferred via : Publications, Disorders (show sections)
3	CHGA	chromogranin A (parathyroid secretory protein 1)	17.14	GeneCards inferred via : Publications (show sections)
4	SMARCB1	SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1	15.14	GeneCards inferred via : Publications (show sections)
5	SIX1	SIX homeobox 1	13.09	GeneCards inferred via : Publications (show sections)
6	B3GAT1	beta-1,3-glucuronyltransferase 1 (glucuronosyltransferase P)	11.09	GeneCards inferred via : Publications, Disorders (show sections)
7	NES	nestin	9.09	GeneCards inferred via : Publications, Disorders (show sections)
8	SOX10	SRY (sex determining region Y)-box 10	7.09	GeneCards inferred via : Publications, Disorders (show sections)
9	ACTC1	actin, alpha, cardiac muscle 1	5.09	GeneCards inferred via : Publications (show sections)
10	HMGA1	high mobility group AT-hook 1	3.09	GeneCards inferred via : Publications (show sections)

Products for genes related to Malignant Peripheral Nerve Sheath Tumor disease:

id	Company	Proteins	Lysates	Antibodies	Kits and Assays
1	Antibodies-online	10	-	10	10
2	Novus Biologicals	9	9	10	-

Variations for Malignant Peripheral Nerve Sheath Tumor

Expression for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
²BioGPS, ¹⁶Gene 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

Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Sources:
⁴⁴Novoseek, ²⁵HMDB, ¹²DrugBank, ²⁹IUPHAR, ⁵⁰PharmGKB, ⁶⁰Tocris Bioscience
See all sources

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

(show all 40)

id	Compound	Score	Top Affiliating Genes
1	l-amino acid⁴⁴	10.1	S100B, CHGA
2	catecholamine⁴⁴	9.9	S100B, CHGA, NGFR
3	orcein⁴⁴	9.7	S100B, ACTC1
4	reticulin⁴⁴	9.7	ACTC1, S100B
5	choline⁴⁴ ²⁵ ¹²	11.7	NGFR, NES, S100B
6	bromodeoxyuridine⁴⁴	9.7	S100B, CHGA, NES
7	mucicarmine⁴⁴	9.6	S100B, ACTC1
8	acth⁴⁴	9.6	B3GAT1, S100B, CHGA
9	streptozotocin⁴⁴	9.6	NES, CHGA
10	c-peptide⁴⁴	9.6	CHGA, NES
11	histamine⁴⁴ ²⁹ ²⁵	11.5	B3GAT1, CHGA, NGFR
12	s 100⁴⁴	9.4	S100B, ACTC1, CHGA
13	methanol⁴⁴ ²⁵	10.4	CHGA, ACTC1
14	forskolin⁴⁴ ⁵⁰ ¹²	11.4	S100B, CHGA, NGFR
15	b 723⁴⁴	9.4	S100B, ACTC1, CHGA
16	paclitaxel⁴⁴ ⁵⁰ ¹²	11.4	SOX10, CHGA, S100B
17	gaba⁴⁴	9.4	NGFR, NES, CHGA, S100B
18	acetylcholine⁴⁴ ⁵⁰ ²⁹ ²⁵ ¹²	13.4	NGFR, NES, CHGA, S100B
19	dopamine⁴⁴ ²⁹ ²⁵ ¹²	12.3	NGFR, NES, CHGA, S100B
20	formaldehyde⁴⁴ ²⁵	10.3	S100B, ACTC1, CHGA
21	glutamate⁴⁴	9.2	S100B, CHGA, NES, NGFR
22	hematoxylin⁴⁴	9.1	NGFR, CHGA, ACTC1, S100B
23	arginine⁴⁴	9.1	CHGA, S100B, HMGA1, B3GAT1
24	glycogen⁴⁴ ²⁵	10.1	S100B, ACTC1, CHGA
25	fatty acid⁴⁴	9.0	HMGA1, S100B, CHGA
26	dexamethasone⁴⁴ ⁵⁰ ²⁹ ¹²	11.9	NGFR, CHGA, ACTC1, S100B
27	nitric oxide⁴⁴ ²⁵ ¹²	10.9	B3GAT1, S100B, CHGA, NES, NGFR
28	testosterone⁴⁴ ⁶⁰ ²⁵ ¹²	11.8	CHGA, ACTC1, S100B, B3GAT1
29	lipid⁴⁴	8.8	HMGA1, S100B, CHGA, NES, NGFR
30	paraffin⁴⁴	8.6	NGFR, NES, CHGA, ACTC1, S100B
31	steroid⁴⁴	8.6	HMGA1, S100B, ACTC1, CHGA, NGFR
32	vegf⁴⁴	8.6	NGFR, NES, CHGA, ACTC1, S100B
33	oxygen⁴⁴ ²⁵	9.5	NGFR, NES, CHGA, ACTC1, S100B
34	matrigel⁴⁴	8.4	NGFR, NES, CHGA, ACTC1, HMGA1
35	progesterone⁴⁴ ²⁹ ⁶⁰ ²⁵ ¹²	12.1	NGFR, NES, CHGA, ACTC1, S100B, B3GAT1
36	retinoic acid⁴⁴ ²⁵	9.0	NGFR, NES, CHGA, ACTC1, S100B, HMGA1
37	glucose⁴⁴	8.0	NGFR, NES, CHGA, ACTC1, S100B, HMGA1
38	calcium⁴⁴ ⁵⁰ ²⁵ ¹²	11.0	NGFR, CHGA, ACTC1, S100B, HMGA1, B3GAT1
39	tyrosine⁴⁴	8.0	NGFR, NES, CHGA, ACTC1, S100B, HMGA1
40	estrogen⁴⁴	7.6	B3GAT1, NGFR, NES, CHGA, ACTC1, S100B