MCID: EPT008
MIFTS: 26

Epithelioid Malignant Peripheral Nerve Sheath Tumor

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards integrated aliases for Epithelioid Malignant Peripheral Nerve Sheath Tumor:

Name: Epithelioid Malignant Peripheral Nerve Sheath Tumor 12 15 17 70
Malignant Epithelioid Neoplasm of the Peripheral Nerve Sheath 12
Epithelioid Mpnst 12

Classifications:



External Ids:

Disease Ontology 12 DOID:8353
NCIt 50 C6561
SNOMED-CT 67 253093000
UMLS 70 C1321427

Summaries for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards based summary : Epithelioid Malignant Peripheral Nerve Sheath Tumor, also known as malignant epithelioid neoplasm of the peripheral nerve sheath, is related to chordoma and embryonal sarcoma. An important gene associated with Epithelioid Malignant Peripheral Nerve Sheath Tumor is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1). Affiliated tissues include kidney, skin and heart, and related phenotype is integument.

Related Diseases for Epithelioid Malignant Peripheral Nerve Sheath Tumor

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

(show top 50) (show all 256)
# Related Disease Score Top Affiliating Genes
1 chordoma 30.6 SMARCB1 S100B MUC1
2 embryonal sarcoma 30.6 S100B MYOG
3 medulloepithelioma 30.6 SMARCB1 MUC1
4 neurilemmomatosis 30.4 SOX10 SMARCB1 NF1
5 epithelioid sarcoma 30.2 SMARCB1 MUC1 CD34
6 angiosarcoma 30.1 MUC1 MLANA CD34
7 neurilemmoma 29.9 SOX10 SMARCB1 S100B NF1 MUC1 CD34
8 gastrointestinal stromal tumor 29.7 SMARCB1 S100B NF1 CHGA CD34
9 rhabdoid cancer 29.7 SSX2 SS18 SMARCB1 MYOG MUC1
10 mesenchymal cell neoplasm 29.5 SSX2 MYOG MLANA CD34
11 ewing sarcoma 29.4 TLE1 SS18 S100B NF1 MYOG CHGA
12 malignant peripheral nerve sheath tumor 29.3 TLE1 SOX10 SMARCB1 S100B NF1 MYOG
13 neurofibroma 29.3 SOX10 S100B NF1 MUC1 MLANA CD34
14 neurofibromatosis 10.6
15 soft tissue sarcoma 10.5
16 melanoma 10.4
17 primary hepatic neuroendocrine carcinoma 10.4 S100B CHGA
18 sclerosing perineurioma 10.4 S100B MUC1
19 localized hypertrophic neuropathy 10.4 S100B MUC1
20 reticular perineurioma 10.4 S100B MUC1
21 intracranial cysts 10.4 S100B MUC1
22 aggressive digital papillary adenocarcinoma 10.4 S100B MUC1
23 eccrine porocarcinoma 10.4 S100B MUC1
24 cerebral primitive neuroectodermal tumor 10.4 S100B MUC1
25 pineocytoma 10.4 S100B CHGA
26 laryngeal neuroendocrine tumor 10.3 SMARCB1 CHGA
27 non-functioning pituitary adenoma 10.3 S100B MUC1
28 esophageal neuroendocrine tumor 10.3 MUC1 CHGA
29 vulvar eccrine porocarcinoma 10.3 MUC1 CHGA
30 middle ear adenoma 10.3 MUC1 CHGA
31 lentigo maligna melanoma 10.3 S100B MLANA
32 syringocystadenoma papilliferum 10.3 S100B MUC1
33 chondroid chordoma 10.3 SMARCB1 MUC1
34 anaplastic ependymoma 10.3 S100B MUC1
35 parachordoma 10.3 MUC1 CHGA
36 sensory organ benign neoplasm 10.3 MLANA CHGA
37 solid adenocarcinoma with mucin production 10.3 MUC1 CHGA
38 ossifying fibromyxoid tumor 10.3 S100B MUC1
39 melanotic neurilemmoma 10.3 SMARCB1 MLANA
40 polymorphous low-grade adenocarcinoma 10.3 S100B MUC1
41 optic nerve astrocytoma 10.3 SMARCB1 NF1
42 chordoid glioma 10.3 S100B MUC1
43 peripheral nerve schwannoma 10.3 SMARCB1 NF1
44 dendritic cell tumor 10.3 S100B MUC1
45 secretory meningioma 10.3 SMARCB1 MUC1
46 optic nerve sheath meningioma 10.3 SMARCB1 NF1
47 spiradenoma 10.3 S100B MUC1
48 optic nerve neoplasm 10.3 SMARCB1 NF1
49 corpus callosum lipoma 10.3 SMARCB1 NF1
50 cerebral hemisphere lipoma 10.3 SMARCB1 NF1

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



Diseases related to Epithelioid Malignant Peripheral Nerve Sheath Tumor

Symptoms & Phenotypes for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MGI Mouse Phenotypes related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.23 CD34 MLANA MYOG NF1 PRG2 S100B

Drugs & Therapeutics for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Search Clinical Trials , NIH Clinical Center for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Genetic Tests for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Anatomical Context for Epithelioid Malignant Peripheral Nerve Sheath Tumor

MalaCards organs/tissues related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

40
Kidney, Skin, Heart, Small Intestine, Liver, Lung, Thyroid

Publications for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Articles related to Epithelioid Malignant Peripheral Nerve Sheath Tumor:

(show all 45)
# Title Authors PMID Year
1
Cytomorphologic Spectrum of SMARCB1-Deficient Soft Tissue Neoplasms. 61
33608696 2021
2
A Rare Case of Epithelioid Malignant Peripheral Nerve Sheath Tumor Mimicking Malignant Melanoma. 61
33763318 2021
3
A case of INI1-deficient tumor in the forearm successfully diagnosed as epithelioid malignant peripheral nerve sheath tumor: Intratumoral nerve fibers with Wallerian degeneration as a diagnostic aid. 61
33559197 2021
4
Vaginal epithelioid malignant peripheral nerve sheath tumor nearly misdiagnosed as advanced cervical cancer: A case report. 61
33360976 2021
5
A rare case of malignant vagus nerve sheath tumor presenting with multiple cranial nerve dysfunction in a dog. 61
33012818 2020
6
SWI/SNF complex-deficient soft tissue neoplasms: An update. 61
32646614 2020
7
Primary pulmonary epithelioid malignant peripheral nerve sheath tumor in a child: a case report. 61
32355847 2020
8
Epithelioid malignant peripheral nerve sheath tumor arising in schwannoma. 61
32913618 2020
9
Recurrent epithelioid malignant peripheral nerve sheath tumor with neurofibromatosis type 1: A case report and literature review. 61
31452784 2019
10
[Epithelioid malignant peripheral nerve sheath tumor: report of a case]. 61
31422602 2019
11
Epithelioid Malignant Peripheral Nerve Sheath Tumor (Epithelioid-MPNST) Presenting as Bleeding Rectal Polyp: A Case Report with Systematic Literature Review. 61
31399553 2019
12
SMARCB1-deficient Tumors of Childhood: A Practical Guide. 61
29280680 2018
13
SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases. 61
28368924 2017
14
Clinicopathologic features and immunohistochemical spectrum of 11 cases of epithelioid malignant peripheral nerve sheath tumors, including INI1/SMARCB1 results and BRAF V600E analysis. 61
28452074 2017
15
Metastatic Epithelioid Malignant Peripheral Nerve Sheath Tumor in a Known Case of Neurofibromatosis-1, Cytomorphological Appearance, and Critical Analysis of Immunohistochemistry. 61
29200699 2017
16
Fine-needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature. 61
26646298 2016
17
Secondary Neoplasms in Children with Hodgkin's Lymphoma Receiving C-MOPP and Radiotherapy: Presentation of Four Cases. 61
26377258 2016
18
Diagnostically Challenging Epithelioid Soft Tissue Tumors. 61
26297059 2015
19
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. 61
25602794 2015
20
A misdiagnosed melanoma: a case of cutaneous epithelioid malignant peripheral nerve sheath tumor. 61
25933073 2015
21
Huge epithelioid malignant peripheral nerve sheath tumor in the left axilla: a case report. 61
26366361 2015
22
[Metastases to the breast from non-mammary malignancies: a clinicopathologic study of 28 cases]. 61
24915812 2014
23
Selected topics in the pathology of epithelioid soft tissue tumors. 61
24384854 2014
24
Conventional spindle cell-type malignant peripheral nerve sheath tumor arising in a sporadic schwannoma. 61
24007690 2013
25
Rapidly progressive epithelioid malignant peripheral nerve sheath tumor of the vestibular nerve. 61
23988994 2013
26
Clinicopathological features, diagnosis and treatment of clear cell sarcoma/melanoma of soft parts. 61
25031505 2013
27
Sporadic superficial diffuse neurofibromas with repeated local recurrence over many years and a tendency toward malignant change: a report of 3 cases. 61
23648456 2013
28
Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with "neuroblastoma-like" schwannomatosis and a novel germline SMARCB1 mutation. 61
22082606 2012
29
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. 61
20952276 2011
30
Epithelioid Lesions. 61
26837652 2011
31
Advanced epithelioid malignant peripheral nerve sheath tumor showing complete response to combined surgery and chemotherapy: a case report. 61
22606446 2011
32
Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease. 61
17972308 2008
33
Epithelioid malignant peripheral nerve sheath tumor in the infraorbital nerve. 61
16550070 2006
34
Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts. 61
16220298 2006
35
Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus. 61
16382783 2005
36
Epithelioid malignant peripheral nerve sheath tumor. Report of a case with inflammatory infiltration. 61
15991844 2005
37
Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases. 61
15592781 2004
38
[Epithelioid malignant peripheral nerve sheath tumor. A case report]. 61
15107669 2004
39
Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. 61
11145248 2001
40
Epithelioid variant of malignant peripheral nerve sheath tumor (malignant schwannoma) of the urinary bladder. 61
10556478 1999
41
Intraosseous epithelioid malignant peripheral nerve sheath tumor of the phalanx. Case report. 61
10230694 1999
42
Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). 61
9285192 1997
43
"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. 61
9042279 1997
44
Purely epithelioid malignant peripheral nerve sheath tumor of the vulva. 61
9142667 1997
45
The purely epithelioid malignant peripheral nerve sheath tumor. 61
2425646 1986

Variations for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Expression for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Search GEO for disease gene expression data for Epithelioid Malignant Peripheral Nerve Sheath Tumor.

Pathways for Epithelioid Malignant Peripheral Nerve Sheath Tumor

GO Terms for Epithelioid Malignant Peripheral Nerve Sheath Tumor

Cellular components related to Epithelioid Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 SWI/SNF complex GO:0016514 8.96 SS18 SMARCB1
2 npBAF complex GO:0071564 8.62 SS18 SMARCB1

Biological processes related to Epithelioid Malignant Peripheral Nerve Sheath Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peripheral nervous system development GO:0007422 8.96 SOX10 NF1
2 positive regulation of histone H4 acetylation GO:0090240 8.62 SMARCB1 MUC1

Sources for Epithelioid Malignant Peripheral Nerve Sheath Tumor

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
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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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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