MCID: PLX001
MIFTS: 30

Plexiform Schwannoma

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Plexiform Schwannoma

MalaCards integrated aliases for Plexiform Schwannoma:

Name: Plexiform Schwannoma 12 6 15 70
Plexiform Neurilemmoma 12
Plexiform Neurinoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:3206
NCIt 50 C6969
SNOMED-CT 67 404025004
UMLS 70 C1370659

Summaries for Plexiform Schwannoma

MalaCards based summary : Plexiform Schwannoma, also known as plexiform neurilemmoma, is related to plexiform neurofibroma and secretory meningioma. 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 is Melanocyte Development and Pigmentation. Affiliated tissues include breast, tongue and small intestine, and related phenotypes are Increased proliferation 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 240)
# Related Disease Score Top Affiliating Genes
1 plexiform neurofibroma 30.7 NF2 NF1 KIT
2 secretory meningioma 30.5 SMARCB1 NF2
3 neurilemmomatosis 30.4 SOX10 SMARCB1 NF2 NF1
4 rare tumor 30.4 NF2 KIT
5 neurofibroma 30.0 SOX10 NF2 NF1 KIT CD34
6 neurilemmoma 29.9 SOX10 SMARCB1 NF2 NF1 KIT ENO2
7 lipomatosis, multiple 29.7 NF1 KIT CD34
8 hemangioma 29.6 KIT ENO2 CD34
9 neurofibromatosis, type i 29.4 NF2 NF1 KIT ENO2 CD34
10 cellular schwannoma 29.4 SOX10 SMARCB1 NF2 NF1 CD34
11 malignant peripheral nerve sheath tumor 29.2 SOX10 SMARCB1 NF2 NF1 KIT CD34
12 meningioma, familial 28.9 SOX10 SMARCB1 NF2 NF1 DSP CD34
13 neurofibromatosis 10.6
14 monosomy 22 10.3 SMARCB1 NF2
15 melanotic neurilemmoma 10.3 SMARCB1 NF2
16 meningioma, radiation-induced 10.3 SMARCB1 NF2
17 central nervous system angiosarcoma 10.3 SMARCB1 CD34
18 schwannomatosis 1 10.3 SMARCB1 NF2
19 neurilemmoma of the fifth cranial nerve 10.3 NF2 NF1
20 trigeminal nerve neoplasm 10.3 NF2 NF1
21 polyp of corpus uteri 10.3 NF2 NF1
22 vulvar sarcoma 10.3 SMARCB1 CD34
23 parasagittal meningioma 10.2 NF2 CD34
24 meninges hemangiopericytoma 10.2 NF2 CD34
25 epidural spinal canal neoplasm 10.2 NF2 CD34
26 angiomatous meningioma 10.2 NF2 CD34
27 mechanical ectropion 10.2 NF1 CD34
28 rete ovarii adenoma 10.2 NF2 NF1
29 benign peripheral nerve sheath tumor 10.2
30 rete ovarii benign neoplasm 10.2 NF2 NF1
31 subungual glomus tumor 10.2 NF1 CD34
32 central epithelioid sarcoma 10.2 SMARCB1 CD34
33 epithelioid sarcoma 10.2 SMARCB1 CD34
34 vulvar proximal-type epithelioid sarcoma 10.2 SMARCB1 CD34
35 intraneural perineurioma 10.2 SOX10 NF2
36 large cell carcinoma with rhabdoid phenotype 10.2 SMARCB1 ENO2
37 fibrous meningioma 10.2 NF2 CD34
38 benign ependymoma 10.2 NF2 NF1
39 kidney rhabdoid cancer 10.2 SMARCB1 ENO2
40 adult xanthogranuloma 10.2 NF1 CD34
41 cerebrovascular benign neoplasm 10.2 SMARCB1 ENO2
42 neurofibromatosis, type ii 10.2
43 paresthesia 10.2
44 olfactory nerve neoplasm 10.2 SMARCB1 ENO2
45 cranial nerve malignant neoplasm 10.2 SMARCB1 ENO2
46 pineal gland cancer 10.2 SMARCB1 ENO2
47 central nervous system primitive neuroectodermal neoplasm 10.2 SMARCB1 ENO2
48 penis sarcoma 10.2 ENO2 CD34
49 spinal cord oligodendroglioma 10.2 NF1 ENO2
50 skin granular cell tumor 10.2 NF1 ENO2

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:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.62 NF2 SMARCB1

MGI Mouse Phenotypes related to Plexiform Schwannoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10 CD34 DSP ENO2 KIT NF1 SMARCB1
2 integument MP:0010771 9.87 CD34 DSP KIT NF1 NF2 SMARCB1
3 embryo MP:0005380 9.85 DSP KIT NF1 NF2 SMARCB1 SOX10
4 craniofacial MP:0005382 9.83 DSP ENO2 KIT NF1 NF2
5 digestive/alimentary MP:0005381 9.8 DSP KIT NF1 SMARCB1 SOX10
6 muscle MP:0005369 9.65 DSP KIT NF1 SMARCB1 VGLL2
7 neoplasm MP:0002006 9.63 CD34 KIT NF1 NF2 SMARCB1 SOX10
8 nervous system MP:0003631 9.5 DSP ENO2 KIT NF1 NF2 SMARCB1
9 respiratory system MP:0005388 9.02 ENO2 KIT NF1 NF2 SOX10

Drugs & Therapeutics for Plexiform Schwannoma

Search Clinical Trials , NIH Clinical Center for Plexiform Schwannoma

Genetic Tests for Plexiform Schwannoma

Anatomical Context for Plexiform Schwannoma

MalaCards organs/tissues related to Plexiform Schwannoma:

40
Breast, Tongue, Small Intestine, Colon, Trachea

Publications for Plexiform Schwannoma

Articles related to Plexiform Schwannoma:

(show top 50) (show all 119)
# Title Authors PMID Year
1
Plexiform Schwannoma of the Oral Cavity: Report of Eight Cases and a Review of the Literature. 61
32270393 2021
2
Review of Pediatric Head and Neck Neoplasms that Raise the Possibility of a Cancer Predisposition Syndrome. 61
33723760 2021
3
Paraspinal plexiform schwannoma of unknown nerve origin: A case report. 61
33486308 2021
4
Visceral plexiform schwannoma: A case series. 61
33282289 2021
5
Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles. 61
33337633 2020
6
Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review. 61
32652976 2020
7
Intramandibular plexiform schwannoma presenting as a nonspecific benign lesion: How specific can we be? A case report with a review of the literature. 61
32719282 2020
8
Diagnosis of plexiform schwannoma of the foot in a 66-year-old male: a case report and literature review. 61
33179006 2020
9
[Schwannoma of the sciatic nerve: a case report]. 61
33552351 2020
10
Plexiform schwannomas of cavum conchae in a young girl: report of a rare case. 61
33165427 2020
11
Giant plexiform schwannoma in the plantar aspect of the foot: a case report. 61
31824642 2019
12
Lower Lip Plexiform Schwannoma: Report of a Rare Case and a Literature Review. 61
31543538 2019
13
Plexiform Schwannoma of the Penis: A Rare Subtype of Genital Schwannoma. 61
31073418 2019
14
Oral plexiform schwannoma: A case report and relevant immunohistochemical investigation. 61
30911392 2019
15
Unusual Etiology of Chronic Posterior Leg Pain in a Running Athlete: Could It Be a Schwannoma? A Case Report and Review of the Literature. 61
31781450 2019
16
Sudden-onset C8 Radiculopathy due to a Plexiform Schwannoma of the Cervical Nerve Root. 61
29984772 2018
17
Plexiform Schwannoma with Localized Hypertrichosis. 61
30065605 2018
18
Magnetic resonance imaging findings for differential diagnosis of perianal plexiform schwannoma: Case report and review of the literature. 61
29774221 2018
19
Recurrent plexiform schwannoma involving the carotid canal. 61
28487042 2018
20
Plexiform Schwannoma of the Tongue in a Pediatric Patient with Neurofibromatosis Type 2: A Case Report and Review of Literature. 61
30410810 2018
21
Low-fat, plexiform spindle cell lipoma of the lip expressing S100 protein: a neural tumor simulator. 61
29940654 2018
22
Plexiform Schwannoma of the Finger: A Case Report and Literature Review. 61
28979871 2017
23
Percutaneous Image-Guided Cryotherapy for Local Control of Recurrent Plexiform Schwannoma in a 3-Year-Old Male. 61
28431654 2017
24
Plexiform schwannoma of the thoracolumbar spine a rare clinical entity - a case report. 61
27809595 2017
25
Plexiform schwannoma: an unusual clitoral mass. 61
27818132 2017
26
Gastric plexiform schwannoma in association with neurofibromatosis type 2. 61
27696205 2016
27
Morphological spectrum of peripheral nerve sheath tumors: An insight into World Health Organization 2013 classification. 61
27365950 2016
28
Congenital cellular plexiform schwannoma mimicking a vascular lesion: Potential pitfalls in clinical and histopathological assessment. 61
26728820 2016
29
Oral plexiform schwannoma with unusual epithelial induction. 61
26268670 2015
30
A rare case of plexiform schwannoma of the lower lip: Treatment and management. 61
26424990 2015
31
Plexiform Schwannoma of Lumbar Region. 61
26064806 2015
32
Plexiform schwannoma of the posterior pharyngeal wall in a patient with neurofibromatosis 2. 61
25738721 2015
33
Successful enucleation of large multinodular/plexiform schwannoma of the foot and ankle. 61
26090307 2015
34
Plexiform schwannoma involving the trachea and recurrent laryngeal nerve: a case report. 61
26366364 2015
35
Plexiform schwannoma arising from cauda equina. 61
25264320 2015
36
[1 case of vocal cord plexiform schwannoma]. 61
25980170 2014
37
Multiple plexiform schwannomas in the plantar aspect of the foot: case report and literature review. 61
25306531 2014
38
A hybrid schwannoma of median nerve. 61
24717998 2014
39
Case presentations and review of plexiform schwannoma in the foot. 61
24556484 2014
40
Plexiform schwannoma of the rectum. 61
23363231 2014
41
Multinodular/plexiform schwannoma of the ulnar nerve. 61
24165375 2014
42
Plexiform schwannoma of the clitoris in a young girl: a case report. 61
23947092 2013
43
Unusual intramaxillary plexiform schwannoma. 61
22847037 2013
44
Macrodactyly in the setting of a plexiform schwannoma in neurofibromatosis type 2: case report. 61
23453897 2013
45
Neuroimage: giant plexiform schwannoma of the penis. 61
23207604 2013
46
Plexiform hypoglossal schwannoma of the tongue and the submandibular region. 61
22976661 2012
47
Plexiform schwannoma of the duodenum accompanying pyloric stenosis: report of a case. 61
22965512 2012
48
Recurrent orbital schwannomas: clinical course and histopathologic correlation. 61
22937797 2012
49
Giant congenital nevus with plexiform neurofibroma and malignant peripheral nerve sheath tumor. 61
22413842 2012
50
Plexiform (multinodular) schwannoma of soft palate. Report of a case. 61
23270209 2012

Variations for Plexiform Schwannoma

ClinVar genetic disease variations for Plexiform Schwannoma:

6
# 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 10.24 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 visual learning GO:0008542 9.37 NF1 KIT
2 pigmentation GO:0043473 9.32 NF1 KIT
3 stem cell differentiation GO:0048863 9.26 SOX10 KIT
4 negative regulation of MAPK cascade GO:0043409 9.16 NF2 NF1
5 peripheral nervous system development GO:0007422 8.96 SOX10 NF1
6 melanocyte differentiation GO:0030318 8.62 SOX10 KIT

Sources for Plexiform Schwannoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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