MCID: ECT002
MIFTS: 32

Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Ectomesenchymoma

MalaCards integrated aliases for Ectomesenchymoma:

Name: Ectomesenchymoma 12 20 15
Rhabdomyosarcoma with Ganglionic Differentiation 12 70
Malignant Ectomesenchymoma 20

Classifications:



External Ids:

Disease Ontology 12 DOID:4055
NCIt 50 C4716
SNOMED-CT 67 128750008
UMLS 70 C0431111

Summaries for Ectomesenchymoma

MalaCards based summary : Ectomesenchymoma, also known as rhabdomyosarcoma with ganglionic differentiation, is related to neurilemmoma and atypical teratoid rhabdoid tumor. An important gene associated with Ectomesenchymoma is ENO2 (Enolase 2), and among its related pathways/superpathways are Transcriptional misregulation in cancer and Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include pineal, tongue and eye, and related phenotypes are Increased Nanog expression and Increased Nanog expression

Wikipedia : 73 Ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that occurs mainly... more...

Related Diseases for Ectomesenchymoma

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

(show top 50) (show all 138)
# Related Disease Score Top Affiliating Genes
1 neurilemmoma 30.2 S100A1 ENO2 DES
2 atypical teratoid rhabdoid tumor 29.7 S100A1 HMGA2 DES
3 liposarcoma 29.6 MYOG HMGA2 DES
4 malignant peripheral nerve sheath tumor 29.6 S100A1 MYOG HMGA2
5 embryonal rhabdomyosarcoma 29.6 PAX3 MYOG HRAS FOXO1 DES
6 rhabdomyosarcoma 2 29.2 PAX3 MYOG FOXO1 ENO2 DES
7 medulloblastoma 29.1 PAX3 HRAS FOXO1 ENO2 DES
8 leiomyosarcoma 29.0 S100A1 MYOG HMGA2 ENO2 DES
9 ewing sarcoma 28.3 PAX3 MYOG FOXO1 ENO2 DES CD99
10 rhabdomyosarcoma 28.3 S100A1 PAX3 MYOG HRAS HMGA2 FOXO1
11 primitive neuroectodermal tumor of the cervix uteri 10.3 ENO2 DES
12 spindle cell thymoma 10.3 ENO2 DES
13 congenital epulis 10.3 ENO2 DES
14 epulis 10.3 ENO2 DES
15 gangliocytoma 10.2
16 ganglioneuroma 10.2
17 rare tumor 10.2
18 ring chromosome 1 10.2 HMGA2 HMGA1
19 ring chromosome 10.2 HMGA2 HMGA1
20 pelvic lipomatosis 10.2 HMGA2 HMGA1
21 intravenous leiomyomatosis 10.2 HMGA2 DES
22 ampulla of vater benign neoplasm 10.2 HRAS ENO2
23 central nervous system melanocytic neoplasm 10.2 HRAS ENO2
24 ovarian small cell carcinoma 10.2 ENO2 DES
25 papillary tumor of the pineal region 10.2 S100A1 ENO2
26 epithelioid leiomyosarcoma 10.2 ENO2 DES
27 central nervous system rhabdomyosarcoma 10.2 PAX3 FOXO1
28 ganglioneuroblastoma 10.1
29 neuroblastoma 10.1
30 conventional fibrosarcoma 10.1 PAX3 ENO2
31 pineal region teratoma 10.1 MYOG ENO2
32 ovarian benign neoplasm 10.1 HRAS DES
33 eccrine papillary adenoma 10.1 HRAS HMGA2
34 parachordoma 10.1 S100A1 DES
35 extracranial neuroblastoma 10.1 MYOG ENO2
36 cervical neuroblastoma 10.1 MYOG ENO2
37 glomangioma 10.1 S100A1 DES
38 myoblastoma 10.1 MYOG ENO2
39 reticulum cell sarcoma 10.1 S100A1 DES
40 endobronchial leiomyoma 10.1 MYOG ENO2
41 neurofibrosarcoma 10.1 S100A1 ENO2
42 uterus leiomyosarcoma 10.1 MYOG ENO2
43 ischemic fasciitis 10.1 S100A1 DES
44 inflammatory leiomyosarcoma 10.1 MYOG DES
45 gallbladder sarcoma 10.1 MYOG DES
46 chondromyxoid fibroma 10.1 S100A1 DES
47 epithelial-myoepithelial carcinoma 10.1 HRAS HMGA2
48 soft tissue sarcoma 10.1
49 rare soft tissue tumor 10.1
50 cervical polyp 10.1 MYOG DES

Graphical network of the top 20 diseases related to Ectomesenchymoma:



Diseases related to Ectomesenchymoma

Symptoms & Phenotypes for Ectomesenchymoma

GenomeRNAi Phenotypes related to Ectomesenchymoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased Nanog expression GR00371-A-3 8.92 HMGA1 HRAS PAX3
2 Increased Nanog expression GR00371-A-5 8.92 HMGA1

MGI Mouse Phenotypes related to Ectomesenchymoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.63 DES FOXO1 HRAS MYOG PAX3 S100A1
2 no phenotypic analysis MP:0003012 9.35 FOXO1 HMGA1 HRAS MYOG PAX3
3 respiratory system MP:0005388 9.02 ENO2 HRAS MYOG PAX3 S100A1

Drugs & Therapeutics for Ectomesenchymoma

Search Clinical Trials , NIH Clinical Center for Ectomesenchymoma

Genetic Tests for Ectomesenchymoma

Anatomical Context for Ectomesenchymoma

MalaCards organs/tissues related to Ectomesenchymoma:

40
Pineal, Tongue, Eye, Lymph Node, Medulla Oblongata, Bone, Heart

Publications for Ectomesenchymoma

Articles related to Ectomesenchymoma:

(show top 50) (show all 73)
# Title Authors PMID Year
1
Primary uterine ectomesenchymoma harboring a DICER1 mutation: case report with molecular analysis. 61
33595736 2021
2
Infant malignant ectomesenchymoma masquerading as inguinal hernia in two patients. 61
33648897 2021
3
More Than Meets the Eye? A Cautionary Tale of Malignant Ectomesenchymoma Treated as Low-risk Orbital Rhabdomyosarcoma. 61
32769567 2020
4
Epidemiology, Incidence, and Survival of Rhabdomyosarcoma Subtypes: SEER and ICES Database Analysis. 61
31161653 2019
5
Malignant Ectomesenchymoma: A Potential Pitfall of Diagnosis in the Spectrum of Pediatric Small Blue Round Cell Tumors. 61
28968263 2019
6
Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature. 61
30907031 2019
7
Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor. 61
28994342 2018
8
Robot-assisted retroperitoneal lymph node dissection (RA-RPLND) in the adolescent population. 61
28262537 2017
9
Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma. 61
26872011 2016
10
An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis. 61
26793539 2015
11
A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum. 61
26339431 2015
12
Ectomesenchymoma with embryonal rhabdomyosarcoma and ganglioneuroma, arising in association with benign triton tumor of the tongue. 61
24650353 2014
13
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. 61
25405050 2014
14
Malignant pediatric brain tumor of primitive small round cell proliferation with bland-looking mesenchymal spindle cell elements. 61
22684841 2013
15
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). 61
22535600 2013
16
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. 61
23174743 2012
17
Ectomesenchymoma with t(1;12)(p32;p13) evolving from embryonal rhabdomyosarcoma shows no rearrangement of ETV6. 61
21803398 2012
18
Malignant ectomesenchymoma of the nasal cavity. 61
22563163 2011
19
A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri. 61
21194292 2011
20
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. 61
22026142 2011
21
Retroperitoneal ectomesenchymoma: a rare childhood tumor. 61
20090275 2010
22
[Malignant ectomesenchymoma of left vulva in childhood: report of a case]. 61
20078983 2009
23
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. 61
18043289 2007
24
Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. 61
17895746 2007
25
Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor. 61
17431644 2007
26
Malignant ectomesenchymoma of the nasal cavity. 61
17395109 2007
27
A neonate with malignant ectomesenchymoma. 61
16363352 2005
28
A 10-month-old boy with a large pineal tumor. 61
16196394 2005
29
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. 61
15747131 2005
30
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. 61
15735865 2005
31
Ectomesenchymoma: case report and review of the literature. 61
15074381 2004
32
Malignant ectomesenchymoma of the nose. Case report and review of the literature. 61
12624508 2003
33
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. 61
12469232 2003
34
Rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report. 61
12461409 2002
35
Primary malignant ectomesenchymoma of the kidney. 61
12383226 2002
36
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. 61
12161212 2002
37
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. 61
11787870 2002
38
[Pelvic malignant ectomesenchymoma: a case report]. 61
11685134 2001
39
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. 61
11297080 2001
40
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. 61
10503746 1999
41
Malignant ectomesenchymoma. Case report and review of the literature. 61
10494059 1999
42
Ectomesenchymoma of the prostate: histological diagnostic criteria. 61
9914362 1999
43
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. 61
9544170 1998
44
Immunohistochemical and molecular genetic approaches to soft tissue tumor diagnosis: a primer. 61
9344317 1997
45
Ectomesenchymoma of the medulla oblongata in a calf. 61
9421949 1997
46
Ectomesenchymoma: one or two tumors? Case report and review of the literature. 61
9314261 1997
47
Radiologic-Pathologic Conference of Children's Hospital Boston: intraconal mass in the orbit of an infant. 61
9252435 1997
48
Primary malignant ectomesenchymoma of the orbit. 61
9065587 1997
49
Malignant ectomesenchymoma in childhood. 61
9025858 1996
50
Ectomesenchymal hamartoma (benign "ectomesenchymoma") of the VIIIth nerve: case report. 61
8584163 1995

Variations for Ectomesenchymoma

Expression for Ectomesenchymoma

Search GEO for disease gene expression data for Ectomesenchymoma.

Pathways for Ectomesenchymoma

Pathways related to Ectomesenchymoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.42 PAX3 HMGA2 FOXO1
2 10.78 PAX3 MYOG DES
3 10.63 HRAS FOXO1

GO Terms for Ectomesenchymoma

Cellular components related to Ectomesenchymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleoplasm GO:0005654 9.7 S100A1 PAX3 MYOG HRAS HMGA2 HMGA1
2 chromatin GO:0000785 9.35 PAX3 MYOG HMGA2 HMGA1 FOXO1
3 protein-DNA complex GO:0032993 9.26 MYOG HMGA2
4 senescence-associated heterochromatin focus GO:0035985 8.62 HMGA2 HMGA1

Biological processes related to Ectomesenchymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 regulation of transcription, DNA-templated GO:0006355 9.85 PAX3 MYOG HMGA2 HMGA1 FOXO1
2 positive regulation of transcription, DNA-templated GO:0045893 9.65 PAX3 MYOG HMGA2 HMGA1 FOXO1
3 positive regulation of transcription by RNA polymerase II GO:0045944 9.63 PAX3 MYOG HRAS HMGA2 HMGA1 FOXO1
4 regulation of heart contraction GO:0008016 9.43 S100A1 DES
5 base-excision repair GO:0006284 9.4 HMGA2 HMGA1
6 positive regulation of cell cycle arrest GO:0071158 9.37 MYOG HMGA2
7 positive regulation of cellular senescence GO:2000774 9.32 HMGA2 HMGA1
8 oncogene-induced cell senescence GO:0090402 8.96 HMGA2 HMGA1
9 senescence-associated heterochromatin focus assembly GO:0035986 8.62 HMGA2 HMGA1

Molecular functions related to Ectomesenchymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA-(apurinic or apyrimidinic site) endonuclease activity GO:0003906 9.16 HMGA2 HMGA1
2 AT DNA binding GO:0003680 8.96 HMGA2 HMGA1
3 5'-deoxyribose-5-phosphate lyase activity GO:0051575 8.62 HMGA2 HMGA1

Sources for Ectomesenchymoma

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