MCID: PLX001
MIFTS: 35

Plexiform Schwannoma

Categories: Cancer diseases

Aliases & Classifications for Plexiform Schwannoma

MalaCards integrated aliases 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 neurofibromatosis-noonan syndrome and pediatric fibrosarcoma. 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 Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include breast, small intestine and trachea, 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 150)
id Related Disease Score Top Affiliating Genes
1 neurofibromatosis-noonan syndrome 10.7 NF1 NF2
2 pediatric fibrosarcoma 10.7 NF1 NF2
3 gallbladder papillary carcinoma 10.7 ENO2 NF2
4 ovarian disease 10.7 NF1 NF2
5 borna disease 10.7 NF1 NF2
6 lung lymphoma 10.6 NF1 NF2
7 epilepsy, familial focal, with variable foci 1 10.6 NF1 NF2
8 cervical keratinizing squamous cell carcinoma 10.6 ENO2 NF1
9 enteric pattern testicular yolk sac tumor 10.6 ENO2 NF1
10 cellular neurofibroma 10.5 CD34 DSP
11 tropical spastic paraparesis 10.5 CD34 NF2
12 transitional meningioma 10.5 CD34 NF2
13 glomus tumor 10.5 ENO2 KIT
14 retroperitoneal leiomyosarcoma 10.5 ENO2 KIT
15 thymus small cell carcinoma 10.5 ENO2 KIT
16 sphenoid sinus squamous cell carcinoma 10.5 ENO2 KIT
17 lung leiomyosarcoma 10.4 ENO2 KIT
18 renal adenoma 10.4 CD34 DSP
19 pancreatic agenesis 10.4 ENO2 KIT
20 pineal region yolk sac tumor 10.4 ENO2 NGFR
21 urethral villous adenoma 10.4 ENO2 KIT
22 appendix leiomyoma 10.4 CD34 KIT
23 small intestine leiomyosarcoma 10.4 CD34 KIT
24 liver inflammatory pseudotumor 10.4 CD34 KIT
25 hypersensitivity reaction type iii disease 10.4 ENO2 KIT
26 filamentary keratitis 10.4 CD34 KIT
27 malignant dermis tumor 10.4 CD34 KIT
28 gallbladder leiomyosarcoma 10.4 CD34 KIT
29 testis rhabdomyosarcoma 10.4 CD34 KIT
30 breast leiomyosarcoma 10.4 CD34 KIT
31 breast apocrine carcinoma in situ 10.4 B3GAT1 NF1
32 kidney lipoma 10.4 CD34 NF1
33 pericoronitis 10.4 CD34 KIT
34 fallopian tube squamous cell carcinoma 10.4 CD34 KIT
35 thyroid angiosarcoma 10.4 CD34 KIT
36 gastric cardia adenocarcinoma 10.3 DSP KIT
37 caroli disease 10.3 ENO2 KIT
38 cutaneous liposarcoma 10.3 CD34 KIT
39 anal canal adenocarcinoma 10.3 ENO2 NGFR
40 epicardium cancer 10.3 CD34 NF1
41 lung benign neoplasm 10.3 B3GAT1 ENO2
42 diphtheria 10.3 ENO2 NF1 NF2
43 epidural abscess 10.3 ENO2 NF1 NF2
44 vestibular disease 10.3 ENO2 NF1 NF2
45 fibrosarcoma of bone 10.3 CD34 KIT
46 bone marrow cancer 10.3 ENO2 KIT
47 ossifying fibromyxoid tumor 10.3 CD34 ENO2
48 optic papillitis 10.3 ENO2 KIT
49 iridocyclitis 10.3 CD34 NF1
50 ethmoid sinus schneiderian papilloma 10.3 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 9.95 B3GAT1 ENO2 GFAP KIT NF1 NF2
2 cellular MP:0005384 9.91 CD34 DSP ENO2 GFAP KIT NF1
3 craniofacial MP:0005382 9.8 DSP ENO2 KIT NF1 NF2 NGFR
4 digestive/alimentary MP:0005381 9.65 NF1 NGFR DSP GFAP KIT
5 integument MP:0010771 9.63 CD34 DSP KIT NF1 NF2 NGFR
6 nervous system MP:0003631 9.56 NGFR B3GAT1 DSP ENO2 GFAP KIT
7 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 10)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
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 Anti-Bacterial Agents Phase 2, Phase 3
8 Antibiotics, Antitubercular Phase 2, Phase 3
9 Isophosphamide mustard Phase 2, Phase 3
10 Topoisomerase Inhibitors Phase 2, Phase 3

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
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 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
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
Breast, Small Intestine, Trachea, Tongue

Publications for Plexiform Schwannoma

Articles related to Plexiform Schwannoma:

(show top 50) (show all 66)
id Title Authors Year
1
Recurrent plexiform schwannoma involving the carotid canal. ( 28487042 )
2017
2
Percutaneous Image-Guided Cryotherapy for Local Control of Recurrent Plexiform Schwannoma in a 3-Year-Old Male. ( 28431654 )
2017
3
Gastric plexiform schwannoma in association with neurofibromatosis type 2. ( 27696205 )
2016
4
Plexiform schwannoma of the thoracolumbar spine a rare clinical entity - a case report. ( 27809595 )
2016
5
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. ( 26728820 )
2016
6
Plexiform schwannoma: an unusual clitoral mass. ( 27818132 )
2016
7
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. ( 26658391 )
2015
8
Oral plexiform schwannoma withA unusual epithelial induction. ( 26268670 )
2015
9
Plexiform schwannoma of the posterior pharyngeal wall in a patient with neurofibromatosis 2. ( 25738721 )
2015
10
Plexiform Schwannoma of Lumbar Region. ( 26064806 )
2015
11
Plexiform schwannoma involving the trachea and recurrent laryngeal nerve: a case report. ( 26366364 )
2015
12
A rare case of plexiform schwannoma of the lower lip: Treatment and management. ( 26424990 )
2015
13
Successful enucleation of large multinodular/plexiform schwannoma of the foot and ankle. ( 26090307 )
2015
14
Multinodular/plexiform schwannoma of the ulnar nerve. ( 24165375 )
2014
15
Case presentations and review of plexiform schwannoma in the foot. ( 24556484 )
2014
16
Plexiform schwannoma arising from cauda equina. ( 25264320 )
2014
17
Plexiform schwannoma of the rectum. ( 23363231 )
2014
18
Unusual intramaxillary plexiform schwannoma. ( 22847037 )
2013
19
Plexiform schwannoma of the clitoris in a young girl: a case report. ( 23947092 )
2013
20
Neuroimage: giant plexiform schwannoma of the penis. ( 23207604 )
2013
21
Macrodactyly in the setting of a plexiform schwannoma in neurofibromatosis type 2: case report. ( 23453897 )
2013
22
A rare case of life-threatening giant plexiform schwannoma. ( 22209242 )
2012
23
Plexiform Schwannoma of the nasal tip: surgical approach. ( 22714859 )
2012
24
Plexiform schwannoma of the duodenum accompanying pyloric stenosis: report of a case. ( 22965512 )
2012
25
Multiple plexiform schwannoma of a finger. ( 22266205 )
2012
26
Giant plexiform schwannoma of the tongue. ( 22937374 )
2011
27
Plexiform schwannoma of the foot: a review of the literature and case report. ( 20833568 )
2011
28
Lobular breast carcinoma metastasis to a superficial plexiform schwannoma as the first evidence of an occult breast cancer. ( 22123192 )
2011
29
A giant plexiform schwannoma of the brachial plexus: case report. ( 22044580 )
2011
30
Intraosseus plexiform schwannoma of the mandible: immunohistochemical differential diagnosis. ( 21119430 )
2010
31
Laryngeal plexiform schwannoma as first symptom in a patient with neurofibromatosis type 2. ( 20303213 )
2010
32
Plexiform schwannoma of the lip mucosa. ( 19415817 )
2009
33
Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2. ( 19573680 )
2009
34
Plexiform schwannoma of the posterior tibial nerve: a case report. ( 19918424 )
2009
35
Plexiform schwannoma in schwannomatosis. ( 19884750 )
2009
36
Congenital plexiform schwannoma of the clitoris. ( 18382807 )
2008
37
Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region. ( 18439936 )
2008
38
Isolated plexiform schwannoma of the hand. ( 21886708 )
2008
39
Cutaneous plexiform schwannoma of the finger not associated with neurofibromatosis. ( 18284268 )
2008
40
Intra-extradural plexiform schwannoma of the cervical spine. ( 17906563 )
2007
41
Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review. ( 17574948 )
2007
42
Plexiform schwannoma mimicking haemangioma: pitfalls in clinical diagnosis and histological interpretation. ( 17714565 )
2007
43
Plexiform schwannoma of scalp: a case report with brief review of literature. ( 18306559 )
2007
44
Plexiform schwannoma of the clitoris. ( 17614860 )
2007
45
Recurrent plexiform schwannoma in vestibular mucosa. ( 16681871 )
2006
46
Contiguous conventional and plexiform schwannomas. Report of two cases. ( 16509508 )
2006
47
Plexiform schwannoma of the ulnar nerve. ( 16019742 )
2005
48
Multiple plexiform schwannoma of the hand and forearm: a long-term follow-up. ( 15950336 )
2005
49
Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. ( 16006798 )
2005
50
MR imaging features of a scalp plexiform schwannoma. ( 14970034 )
2004

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 intermediate filament organization GO:0045109 8.65 DSP
5 negative regulation of cell-matrix adhesion GO:0001953 8.62 NF1 NF2

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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