MCID: PLX001
MIFTS: 31

Plexiform Schwannoma

Categories: Cancer diseases, Neuronal diseases
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Aliases & Classifications for Plexiform Schwannoma

MalaCards integrated aliases for Plexiform Schwannoma:

Name: Plexiform Schwannoma 11 5 14 71
Plexiform Neurilemmoma 11
Plexiform Neurinoma 11

Classifications:



External Ids:

Disease Ontology 11 DOID:3206
NCIt 49 C6969
SNOMED-CT 68 404025004
UMLS 71 C1370659

Summaries for Plexiform Schwannoma

MalaCards based summary: Plexiform Schwannoma, also known as plexiform neurilemmoma, is related to neurofibromatosis-noonan syndrome and neurofibromatosis, type ii. An important gene associated with Plexiform Schwannoma is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1), and among its related pathways/superpathways are Neuroscience and Melanocyte Development and Pigmentation. Affiliated tissues include tongue, small intestine and breast, and related phenotypes are Increased proliferation and growth/size/body region

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 348)
# Related Disease Score Top Affiliating Genes
1 neurofibromatosis-noonan syndrome 30.3 NF1 LZTR1
2 neurofibromatosis, type ii 30.2 SMARCB1 NF2 NF1 LZTR1
3 acoustic neuroma 30.2 SMARCB1 NF2 NF1 LZTR1
4 neurofibroma 30.1 SOX10 NF2 NF1 KIT CD34
5 neurofibromatosis 30.0 SMARCB1 NF2 NF1 LZTR1 KIT
6 full schwannomatosis 29.8 SMARCB1 NF2 LZTR1
7 neurilemmomatosis 29.8 SOX10 SMARCB1 NF2 NF1 LZTR1
8 hemangioma 29.6 KIT ENO2 CD34
9 malignant peripheral nerve sheath tumor 29.6 SOX10 NF2 NF1 KIT CD34
10 plexiform neurofibroma 29.5 SOX10 SMARCB1 NF2 NF1 LZTR1 KIT
11 neurofibromatosis, type i 29.4 NF2 NF1 KIT ENO2 CD34 ANO1
12 lipomatosis, multiple 29.4 NF1 KIT CD34 ANO1
13 peripheral nervous system neoplasm 29.1 NF2 NF1 KIT ENO2 CD34
14 neurilemmoma 29.0 SOX10 SMARCB1 NF2 NF1 LZTR1 KIT
15 cellular schwannoma 28.5 SOX10 SMARCB1 NF2 NF1 KIT CD34
16 meningioma, familial 28.3 SOX10 SMARCB1 NF2 NF1 LZTR1 KIT
17 neuroma 27.8 SMARCB1 NF2 NF1 LZTR1 KIT ENO2
18 posterior pituitary gland neoplasm 10.3 SMARCB1 NF2
19 childhood meningioma 10.3 SMARCB1 NF2
20 pineal region meningioma 10.3 SMARCB1 NF2
21 cerebral falx meningioma 10.3 SMARCB1 NF2
22 parasagittal meningioma 10.3 SMARCB1 NF2
23 cerebral convexity meningioma 10.3 SMARCB1 NF2
24 schwannomatosis 1 10.3 SMARCB1 NF2
25 olfactory groove meningioma 10.3 SMARCB1 NF2
26 cerebellopontine angle tumor 10.3 SMARCB1 NF2
27 anterior cranial fossa meningioma 10.3 SMARCB1 NF2
28 brain meningioma 10.2 SMARCB1 NF2
29 transitional meningioma 10.2 SMARCB1 NF2
30 secretory meningioma 10.2 SMARCB1 NF2
31 central nervous system angiosarcoma 10.2 SMARCB1 CD34
32 benign peripheral nerve sheath tumor 10.2
33 rare tumor 10.2
34 rhabdoid meningioma 10.2 SMARCB1 NF2
35 skull base meningioma 10.2 SMARCB1 NF2
36 spinal cord ependymoma 10.2 SMARCB1 NF2
37 proliferative fasciitis 10.2 SMARCB1 CD34
38 skull base cancer 10.2 SMARCB1 NF2
39 spinal cord astrocytoma 10.2 NF2 NF1
40 central nervous system fibrosarcoma 10.2 NF2 CD34
41 vulvar sarcoma 10.2 SMARCB1 CD34
42 lung meningioma 10.2 NF2 CD34
43 polyp of corpus uteri 10.2 NF2 NF1
44 psammomatous meningioma 10.2 NF2 CD34
45 meninges hemangiopericytoma 10.2 NF2 CD34
46 biphasic synovial sarcoma 10.2 SMARCB1 CD34
47 angiomatous meningioma 10.2 NF2 CD34
48 rete ovarii adenoma 10.2 NF2 NF1
49 rete ovarii benign neoplasm 10.2 NF2 NF1
50 histiocytoid hemangioma 10.2 SMARCB1 CD34

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



Diseases related to Plexiform Schwannoma

Symptoms & Phenotypes for Plexiform Schwannoma

GenomeRNAi Phenotypes related to Plexiform Schwannoma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.62 NF2 SMARCB1

MGI Mouse Phenotypes related to Plexiform Schwannoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.92 ANO1 ENO2 KIT LZTR1 NF1 NF2
2 nervous system MP:0003631 9.91 ANO1 ENO2 KIT NF1 NF2 SMARCB1
3 neoplasm MP:0002006 9.88 CD34 KIT NF1 NF2 SMARCB1 SOX10
4 pigmentation MP:0001186 9.73 KIT NF1 NF2 SOX10
5 digestive/alimentary MP:0005381 9.73 ANO1 KIT NF1 NF2 SMARCB1 SOX10
6 craniofacial MP:0005382 9.55 ENO2 KIT LZTR1 NF1 NF2
7 respiratory system MP:0005388 9.43 ANO1 ENO2 KIT NF1 NF2 SOX10
8 integument MP:0010771 9.17 ANO1 CD34 KIT NF1 NF2 SMARCB1

Drugs & Therapeutics for Plexiform Schwannoma

Search Clinical Trials, NIH Clinical Center for Plexiform Schwannoma

Genetic Tests for Plexiform Schwannoma

Anatomical Context for Plexiform Schwannoma

Organs/tissues related to Plexiform Schwannoma:

MalaCards : Tongue, Small Intestine, Breast, Trachea, Colon, Skin

Publications for Plexiform Schwannoma

Articles related to Plexiform Schwannoma:

(show top 50) (show all 133)
# Title Authors PMID Year
1
Skin lesions in neurofibromatosis type 2: diagnostic and prognostic significance of cutaneous (plexiform) schwannomas. 62
35490384 2022
2
A Case of Multiple Plexiform Schwannomas. 62
36134103 2022
3
A Rare Case of Plexiform Schwannoma of the Little Finger and Its Management: A Case Report. 62
35911312 2022
4
An Assessment and Management of Plexiform Schwannoma of the Third Webspace in a Female: A Case Report. 62
35774689 2022
5
Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma. 62
34855632 2022
6
Pelvic Plexiform Schwannoma on 18F-FDG PET/MRI. 62
34874350 2022
7
A case of appendiceal ganglioneuroma in neurofibromatosis type 1. 62
34581917 2021
8
A case of peripancreatic plexiform schwannoma. 62
34453629 2021
9
Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles. 62
33337633 2021
10
A rare case of plexiform schwannoma on the foot. 62
34084516 2021
11
Review of Pediatric Head and Neck Neoplasms that Raise the Possibility of a Cancer Predisposition Syndrome. 62
33723760 2021
12
Plexiform Schwannoma of the Oral Cavity: Report of Eight Cases and a Review of the Literature. 62
32270393 2021
13
Paraspinal plexiform schwannoma of unknown nerve origin: A case report. 62
33486308 2021
14
Visceral plexiform schwannoma: A case series. 62
33282289 2021
15
Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review. 62
32652976 2020
16
Intramandibular plexiform schwannoma presenting as a nonspecific benign lesion: How specific can we be? A case report with a review of the literature. 62
32719282 2020
17
Extremely rare case of retropharyngeal space benign plexiform schwannoma - Excised through Smith- Robinson Approach. 62
35079447 2020
18
Diagnosis of plexiform schwannoma of the foot in a 66-year-old male: a case report and literature review. 62
33179006 2020
19
[Schwannoma of the sciatic nerve: a case report]. 62
33552351 2020
20
Plexiform schwannomas of cavum conchae in a young girl: report of a rare case. 62
33165427 2020
21
Giant plexiform schwannoma in the plantar aspect of the foot: a case report. 62
31824642 2019
22
Lower Lip Plexiform Schwannoma: Report of a Rare Case and a Literature Review. 62
31543538 2019
23
Unusual Etiology of Chronic Posterior Leg Pain in a Running Athlete: Could It Be a Schwannoma? A Case Report and Review of the Literature. 62
31781450 2019
24
Plexiform Schwannoma of the Penis: A Rare Subtype of Genital Schwannoma. 62
31073418 2019
25
Oral plexiform schwannoma: A case report and relevant immunohistochemical investigation. 62
30911392 2019
26
Sudden-onset C8 Radiculopathy due to a Plexiform Schwannoma of the Cervical Nerve Root. 62
29984772 2018
27
Plexiform Schwannoma with Localized Hypertrichosis. 62
30065605 2018
28
Magnetic resonance imaging findings for differential diagnosis of perianal plexiform schwannoma: Case report and review of the literature. 62
29774221 2018
29
Recurrent plexiform schwannoma involving the carotid canal. 62
28487042 2018
30
Plexiform Schwannoma of the Tongue in a Pediatric Patient with Neurofibromatosis Type 2: A Case Report and Review of Literature. 62
30410810 2018
31
Low-fat, plexiform spindle cell lipoma of the lip expressing S100 protein: a neural tumor simulator. 62
29940654 2018
32
Plexiform Schwannoma of the Finger: A Case Report and Literature Review. 62
28979871 2017
33
Percutaneous Image-Guided Cryotherapy for Local Control of Recurrent Plexiform Schwannoma in a 3-Year-Old Male. 62
28431654 2017
34
Plexiform schwannoma of the thoracolumbar spine a rare clinical entity - a case report. 62
27809595 2017
35
Plexiform schwannoma: an unusual clitoral mass. 62
27818132 2017
36
Gastric plexiform schwannoma in association with neurofibromatosis type 2. 62
27696205 2016
37
Morphological spectrum of peripheral nerve sheath tumors: An insight into World Health Organization 2013 classification. 62
27365950 2016
38
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. 62
26728820 2016
39
Oral plexiform schwannoma with unusual epithelial induction. 62
26268670 2015
40
A rare case of plexiform schwannoma of the lower lip: Treatment and management. 62
26424990 2015
41
Plexiform Schwannoma of Lumbar Region. 62
26064806 2015
42
Plexiform schwannoma of the posterior pharyngeal wall in a patient with neurofibromatosis 2. 62
25738721 2015
43
Plexiform schwannoma arising from cauda equina. 62
25264320 2015
44
Successful enucleation of large multinodular/plexiform schwannoma of the foot and ankle. 62
26090307 2015
45
Plexiform schwannoma involving the trachea and recurrent laryngeal nerve: a case report. 62
26366364 2015
46
[1 case of vocal cord plexiform schwannoma]. 62
25980170 2014
47
Multiple plexiform schwannomas in the plantar aspect of the foot: case report and literature review. 62
25306531 2014
48
A hybrid schwannoma of median nerve. 62
24717998 2014
49
Case presentations and review of plexiform schwannoma in the foot. 62
24556484 2014
50
Plexiform schwannoma of the rectum. 62
23363231 2014

Variations for Plexiform Schwannoma

ClinVar genetic disease variations for Plexiform Schwannoma:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 SMARCB1 NM_003073.5(SMARCB1):c.*82C>T SNV Pathogenic
239481 rs878854600 GRCh37: 22:24176449-24176449
GRCh38: 22:23834262-23834262

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:

# Super pathways Score Top Affiliating Genes
1 11.46 NF2 NF1 ENO2 ANO1
2 10.57 SOX10 KIT

GO Terms for Plexiform Schwannoma

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

# Name GO ID Score Top Affiliating Genes
1 melanocyte differentiation GO:0030318 9.62 SOX10 KIT
2 negative regulation of cell-matrix adhesion GO:0001953 9.56 NF2 NF1
3 mast cell proliferation GO:0070662 9.46 NF1 KIT
4 Schwann cell proliferation GO:0014010 9.26 NF2 NF1
5 negative regulation of Schwann cell proliferation GO:0010626 9.1 SOX10 NF2 NF1

Sources for Plexiform Schwannoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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