MCID: PLX001
MIFTS: 35

Plexiform Schwannoma malady

Categories: Cancer diseases

Aliases & Classifications for Plexiform Schwannoma

Aliases & Descriptions for Plexiform Schwannoma:

Name: Plexiform Schwannoma 12 14 69
Plexiform Neurilemmoma 12
Plexiform Neurinoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3206
NCIt 47 C6969
UMLS 69 C1370659

Summaries for Plexiform Schwannoma

MalaCards based summary : Plexiform Schwannoma, also known as plexiform neurilemmoma, is related to watson syndrome and lateral medullary syndrome. An important gene associated with Plexiform Schwannoma is NF2 (Neurofibromin 2), and among its related pathways/superpathways are Neuroscience and Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Doxorubicin and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include trachea, tongue and breast, and related phenotypes are behavior/neurological and cellular

Related Diseases for Plexiform Schwannoma

Diseases related to Plexiform Schwannoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 121)
id Related Disease Score Top Affiliating Genes
1 watson syndrome 10.3 NF1 NF2
2 lateral medullary syndrome 10.3 NF1 NF2
3 pleural cancer 10.3 NF1 NF2
4 anorectal stricture 10.3 NF1 NF2
5 chromosome 15q11-q13 duplication syndrome 10.2 DSP NF1
6 epilepsy, familial focal, with variable foci 10.2 NF1 NF2
7 childhood vagina botryoid rhabdomyosarcoma 10.2 NF1 NF2
8 brain ependymoma 10.2 ENO2 NF1
9 multiple mucosal neuroma 10.2 CD34 DSP
10 demyelinating disease 10.2 CD34 NF2
11 spinal meninges cancer 10.2 CD34 NF2
12 glomus tumor 10.2 ENO2 KIT
13 neurofibromatosis 10.2
14 larynx leiomyosarcoma 10.2 ENO2 KIT
15 rectum leiomyoma 10.2 ENO2 KIT
16 adult endodermal sinus tumor 10.2 ENO2 NGFR
17 neurofibroma of gallbladder 10.2 CD34 KIT
18 sphenoid sinus squamous cell carcinoma 10.2 ENO2 KIT
19 gallbladder leiomyosarcoma 10.2 CD34 KIT
20 speech disorder 10.2 CD34 KIT
21 fragile x-associated tremor/ataxia syndrome 10.2 ENO2 KIT
22 freemartinism 10.2 CD34 KIT
23 testis sarcoma 10.2 CD34 KIT
24 gastric leiomyosarcoma 10.2 CD34 KIT
25 prostate leiomyosarcoma 10.2 CD34 KIT
26 postgastrectomy syndrome 10.2 B3GAT1 NF1
27 uterus leiomyosarcoma 10.2 CD34 KIT
28 rhabdoid cancer 10.2 CD34 KIT
29 choriocarcinoma of ovary 10.2 CD34 KIT
30 skin angiosarcoma 10.2 CD34 KIT
31 cowden syndrome 3 10.1 KIT NF1
32 colon signet ring adenocarcinoma 10.1 CD34 KIT
33 diphtheria 10.1 ENO2 NF1 NF2
34 epidural abscess 10.1 ENO2 NF1 NF2
35 granulomatous myositis 10.1 ENO2 NF1 NF2
36 alcohol-induced mental disorder 10.1 CD34 KIT
37 pleural lipoma 10.1 CD34 NF1
38 plexopathy 10.1 B3GAT1 ENO2
39 lung mucoepidermoid carcinoma 10.1 KIT NGFR
40 small intestinal sarcoma 10.1 ENO2 KIT
41 bronchitis 10.1 GFAP NF2
42 occlusion precerebral artery 10.1 KIT NF1 NF2
43 seborrheic dermatitis 10.1 KIT NF1 NF2
44 external ear disease 10.1 GFAP NF2
45 neurofibromatosis-noonan syndrome 10.1 KIT NF1
46 myxopapillary ependymoma 10.1 GFAP NF2
47 ossifying fibromyxoid tumor 10.1 CD34 ENO2
48 peripheral osteosarcoma 10.1 B3GAT1 ENO2
49 cranial nerve disease 10.1 ENO2 KIT
50 uterine corpus myxoid leiomyosarcoma 10.1 B3GAT1 ENO2

Graphical network of the top 20 diseases related to Plexiform Schwannoma:



Diseases related to Plexiform Schwannoma

Symptoms & Phenotypes for Plexiform Schwannoma

MGI Mouse Phenotypes related to Plexiform Schwannoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.98 B3GAT1 ENO2 GFAP KIT NF1 NF2
2 cellular MP:0005384 9.95 CD34 DSP ENO2 GFAP KIT NF1
3 craniofacial MP:0005382 9.85 DSP ENO2 KIT NF1 NF2 NGFR
4 integument MP:0010771 9.73 NF1 NF2 NGFR CD34 DSP KIT
5 digestive/alimentary MP:0005381 9.72 DSP GFAP KIT NF1 NGFR
6 nervous system MP:0003631 9.56 B3GAT1 DSP ENO2 GFAP KIT NF1
7 muscle MP:0005369 9.55 GFAP KIT NF1 NGFR DSP
8 respiratory system MP:0005388 9.02 ENO2 KIT NF1 NF2 NGFR

Drugs & Therapeutics for Plexiform Schwannoma

Drugs for Plexiform Schwannoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6 Alkylating Agents Phase 2, Phase 3
7 Topoisomerase Inhibitors Phase 2, Phase 3
8 Anti-Bacterial Agents Phase 2, Phase 3
9 Isophosphamide mustard Phase 2, Phase 3
10 Antibiotics, Antitubercular Phase 2, Phase 3
11 Antineoplastic Agents, Alkylating Phase 2, Phase 3

Interventional clinical trials:


id Name Status NCT ID Phase
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3
2 Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis Unknown status NCT01777451
3 Transformation of Plexiform Neurofibromas to Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1 Recruiting NCT02211768 Early Phase 1
4 Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors Recruiting NCT03141021
5 Natural History Study of Patients With Neurofibromatosis Type I Recruiting NCT00924196

Search NIH Clinical Center for Plexiform Schwannoma

Genetic Tests for Plexiform Schwannoma

Anatomical Context for Plexiform Schwannoma

MalaCards organs/tissues related to Plexiform Schwannoma:

39
Trachea, Tongue, Breast, Small Intestine

Publications for Plexiform Schwannoma

Articles related to Plexiform Schwannoma:

(show top 50) (show all 64)
id Title Authors Year
1
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. ( 26728820 )
2016
2
Gastric plexiform schwannoma in association with neurofibromatosis type 2. ( 27696205 )
2016
3
Plexiform schwannoma of the thoracolumbar spine a rare clinical entity - a case report. ( 27809595 )
2016
4
Plexiform schwannoma: an unusual clitoral mass. ( 27818132 )
2016
5
Oral plexiform schwannoma withA unusual epithelial induction. ( 26268670 )
2015
6
A rare case of plexiform schwannoma of the lower lip: Treatment and management. ( 26424990 )
2015
7
Plexiform Schwannoma of Lumbar Region. ( 26064806 )
2015
8
Successful enucleation of large multinodular/plexiform schwannoma of the foot and ankle. ( 26090307 )
2015
9
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. ( 26658391 )
2015
10
Plexiform schwannoma involving the trachea and recurrent laryngeal nerve: a case report. ( 26366364 )
2015
11
Plexiform schwannoma of the posterior pharyngeal wall in a patient with neurofibromatosis 2. ( 25738721 )
2015
12
Plexiform schwannoma of the rectum. ( 23363231 )
2014
13
Case presentations and review of plexiform schwannoma in the foot. ( 24556484 )
2014
14
Plexiform schwannoma arising from cauda equina. ( 25264320 )
2014
15
Multinodular/plexiform schwannoma of the ulnar nerve. ( 24165375 )
2014
16
Plexiform schwannoma of the clitoris in a young girl: a case report. ( 23947092 )
2013
17
Macrodactyly in the setting of a plexiform schwannoma in neurofibromatosis type 2: case report. ( 23453897 )
2013
18
Unusual intramaxillary plexiform schwannoma. ( 22847037 )
2013
19
Neuroimage: giant plexiform schwannoma of the penis. ( 23207604 )
2013
20
Plexiform Schwannoma of the nasal tip: surgical approach. ( 22714859 )
2012
21
Multiple plexiform schwannoma of a finger. ( 22266205 )
2012
22
Plexiform schwannoma of the duodenum accompanying pyloric stenosis: report of a case. ( 22965512 )
2012
23
A rare case of life-threatening giant plexiform schwannoma. ( 22209242 )
2012
24
Giant plexiform schwannoma of the tongue. ( 22937374 )
2011
25
A giant plexiform schwannoma of the brachial plexus: case report. ( 22044580 )
2011
26
Plexiform schwannoma of the foot: a review of the literature and case report. ( 20833568 )
2011
27
Lobular breast carcinoma metastasis to a superficial plexiform schwannoma as the first evidence of an occult breast cancer. ( 22123192 )
2011
28
Laryngeal plexiform schwannoma as first symptom in a patient with neurofibromatosis type 2. ( 20303213 )
2010
29
Intraosseus plexiform schwannoma of the mandible: immunohistochemical differential diagnosis. ( 21119430 )
2010
30
Plexiform schwannoma of the posterior tibial nerve: a case report. ( 19918424 )
2009
31
Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2. ( 19573680 )
2009
32
Plexiform schwannoma in schwannomatosis. ( 19884750 )
2009
33
Plexiform schwannoma of the lip mucosa. ( 19415817 )
2009
34
Isolated plexiform schwannoma of the hand. ( 21886708 )
2008
35
Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region. ( 18439936 )
2008
36
Congenital plexiform schwannoma of the clitoris. ( 18382807 )
2008
37
Cutaneous plexiform schwannoma of the finger not associated with neurofibromatosis. ( 18284268 )
2008
38
Plexiform schwannoma of scalp: a case report with brief review of literature. ( 18306559 )
2007
39
Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review. ( 17574948 )
2007
40
Plexiform schwannoma of the clitoris. ( 17614860 )
2007
41
Intra-extradural plexiform schwannoma of the cervical spine. ( 17906563 )
2007
42
Plexiform schwannoma mimicking haemangioma: pitfalls in clinical diagnosis and histological interpretation. ( 17714565 )
2007
43
Recurrent plexiform schwannoma in vestibular mucosa. ( 16681871 )
2006
44
Contiguous conventional and plexiform schwannomas. Report of two cases. ( 16509508 )
2006
45
Plexiform schwannoma of the ulnar nerve. ( 16019742 )
2005
46
Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. ( 16006798 )
2005
47
Multiple plexiform schwannoma of the hand and forearm: a long-term follow-up. ( 15950336 )
2005
48
MR imaging features of a scalp plexiform schwannoma. ( 14970034 )
2004
49
Plexiform schwannoma of the small intestine: report of a case. ( 14669089 )
2003
50
Plexiform schwannoma of the forearm. ( 14593503 )
2003

Variations for Plexiform Schwannoma

Expression for Plexiform Schwannoma

Search GEO for disease gene expression data for Plexiform Schwannoma.

Pathways for Plexiform Schwannoma

Pathways related to Plexiform Schwannoma according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.53 ENO2 GFAP NF1 NF2 NGFR
2 11.11 CD34 KIT NGFR

GO Terms for Plexiform Schwannoma

Cellular components related to Plexiform Schwannoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 desmosome GO:0030057 8.96 CDSN DSP
2 cell-cell junction GO:0005911 8.8 CDSN DSP KIT

Biological processes related to Plexiform Schwannoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 regulation of cell proliferation GO:0042127 9.43 KIT NF1 NF2
2 pigmentation GO:0043473 9.32 KIT NF1
3 negative regulation of MAPK cascade GO:0043409 9.16 NF1 NF2
4 negative regulation of cell-matrix adhesion GO:0001953 8.96 NF1 NF2
5 single organismal cell-cell adhesion GO:0016337 8.8 CD34 CDSN DSP

Sources for Plexiform Schwannoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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