MCID: MLG133
MIFTS: 29

Malignant Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Malignant Ectomesenchymoma

MalaCards integrated aliases for Malignant Ectomesenchymoma:

Name: Malignant Ectomesenchymoma 53
Rhabdomyosarcoma with Ganglionic Differentiation 73
Ectomesenchymoma 53

Classifications:



External Ids:

UMLS 73 C0431111

Summaries for Malignant Ectomesenchymoma

MalaCards based summary : Malignant Ectomesenchymoma, also known as rhabdomyosarcoma with ganglionic differentiation, is related to ectomesenchymoma and rhabdomyosarcoma. An important gene associated with Malignant Ectomesenchymoma is ENO2 (Enolase 2), and among its related pathways/superpathways are Telomere Extension by Telomerase and PI3K-AKT-mTOR signaling pathway and therapeutic opportunities. The drugs Vincristine and Lenograstim have been mentioned in the context of this disorder. Affiliated tissues include kidney, lung and skin.

Wikipedia : 76 Malignant ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that... more...

Related Diseases for Malignant Ectomesenchymoma

Diseases in the Ectomesenchymoma family:

Malignant Ectomesenchymoma

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

(show top 50) (show all 61)
# Related Disease Score Top Affiliating Genes
1 ectomesenchymoma 31.4 DES ENO2 FOXO1 HMGA1 HMGA2 HRAS
2 rhabdomyosarcoma 30.4 DES ENO2 FOXO1
3 embryonal rhabdomyosarcoma 29.9 DES FOXO1 HRAS
4 primitive neuroectodermal tumor of the cervix uteri 10.2 DES ENO2
5 epulis 10.2 DES ENO2
6 ossifying fibromyxoid tumor 10.2 DES ENO2
7 epithelioid leiomyosarcoma 10.2 DES ENO2
8 desmoplastic small round cell tumor 10.2 DES ENO2
9 extraskeletal ewing sarcoma 10.1 DES ENO2
10 glomus tumor 10.1 DES ENO2
11 granular cell tumor 10.1 DES ENO2
12 spindle cell lipoma 10.1 DES FOXO1
13 papillary tumor of the pineal region 10.1 ENO2 S100A1
14 congenital epulis 10.1 DES ENO2
15 medullomyoblastoma 10.1 DES ENO2
16 meningeal melanomatosis 10.1 ENO2 HRAS
17 ischemic fasciitis 10.1 DES S100A1
18 chondroid chordoma 10.1 ENO2 S100A1
19 reticulum cell sarcoma 10.1 DES S100A1
20 duodenum cancer 10.1 ENO2 HRAS
21 mixed cell type cancer 10.1 ENO2 HRAS
22 liver angiosarcoma 10.1 ENO2 HRAS
23 chordoma 10.1 DES S100A1
24 adenosquamous lung carcinoma 10.1 ENO2 HRAS
25 neurilemmoma 10.1 DES ENO2
26 pericardial mesothelioma 10.1 ENO2 S100A1
27 alveolar soft part sarcoma 10.1 DES ENO2
28 central nervous system melanocytic neoplasm 10.0 ENO2 HRAS
29 chondromyxoid fibroma 10.0 DES S100A1
30 skin benign neoplasm 10.0 ENO2 HRAS
31 lung benign neoplasm 10.0 ENO2 HRAS
32 mesenchymoma 10.0 DES HMGA2
33 sarcoma 10.0
34 malignant fibroxanthoma 10.0
35 histiocytoma 10.0
36 spindle cell sarcoma 10.0
37 fibrous histiocytoma 10.0
38 muscle cancer 10.0 DES ENO2 FOXO1
39 bile duct adenocarcinoma 10.0 HRAS S100A1
40 angiomyoma 10.0 ENO2 HMGA2
41 rhabdomyosarcoma 2 10.0 DES ENO2 FOXO1
42 dedifferentiated liposarcoma 10.0 DES HMGA2
43 liposarcoma 10.0 DES HMGA2
44 leiomyosarcoma 10.0 DES ENO2 S100A1
45 carcinosarcoma 10.0 DES HRAS
46 medulloblastoma 10.0
47 atypical teratoid rhabdoid tumor 10.0
48 rhabdoid cancer 10.0
49 ganglioneuroma 10.0
50 gastrointestinal stromal tumor 10.0 DES ENO2 S100A1

Graphical network of the top 20 diseases related to Malignant Ectomesenchymoma:



Diseases related to Malignant Ectomesenchymoma

Symptoms & Phenotypes for Malignant Ectomesenchymoma

Drugs & Therapeutics for Malignant Ectomesenchymoma

Drugs for Malignant Ectomesenchymoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 28)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
2
Lenograstim Approved, Investigational Phase 3 135968-09-1
3
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
4
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
5
Etoposide Approved Phase 3 33419-42-0 36462
6
Mesna Approved, Investigational Phase 3 3375-50-6 598
7
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
8
Irinotecan Approved, Investigational Phase 3 100286-90-6, 97682-44-5 60838
9
Ifosfamide Approved Phase 3 3778-73-2 3690
10
Cyclophosphamide Approved, Investigational Phase 3 6055-19-2, 50-18-0 2907
11
Doxil Approved June 1999 Phase 3 31703
12
Camptothecin Experimental Phase 3 7689-03-4
13
Isophosphamide mustard Phase 3 0
14 Nucleic Acid Synthesis Inhibitors Phase 3
15 Immunosuppressive Agents Phase 3
16 topoisomerase I inhibitors Phase 3
17 Etoposide phosphate Phase 3
18 Alkylating Agents Phase 3
19 Topoisomerase Inhibitors Phase 3
20 Antineoplastic Agents, Phytogenic Phase 3
21 Antibiotics, Antitubercular Phase 3
22 Antineoplastic Agents, Alkylating Phase 3
23 Anti-Bacterial Agents Phase 3
24 Antirheumatic Agents Phase 3
25 Anti-Infective Agents Phase 3
26 Adjuvants, Immunologic Phase 3
27 Antimitotic Agents Phase 3
28 Immunologic Factors Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 High-Dose Combination Chemotherapy and Radiation Therapy in Treating Patients With Newly Diagnosed Metastatic Rhabdomyosarcoma or Ectomesenchymoma Unknown status NCT00354744 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;irinotecan hydrochloride;vincristine sulfate

Search NIH Clinical Center for Malignant Ectomesenchymoma

Genetic Tests for Malignant Ectomesenchymoma

Anatomical Context for Malignant Ectomesenchymoma

MalaCards organs/tissues related to Malignant Ectomesenchymoma:

41
Kidney, Lung, Skin, Liver, Cervix, Smooth Muscle, Pineal

Publications for Malignant Ectomesenchymoma

Articles related to Malignant Ectomesenchymoma:

(show all 29)
# Title Authors Year
1
Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor. ( 28994342 )
2017
2
Malignant Ectomesenchymoma: A Potential Pitfall of Diagnosis in the Spectrum of Pediatric Small Blue Round Cell Tumors. ( 28968263 )
2017
3
Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma. ( 26872011 )
2016
4
An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis. ( 26793539 )
2015
5
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. ( 25405050 )
2014
6
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). ( 22535600 )
2013
7
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. ( 23174743 )
2012
8
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. ( 22026142 )
2011
9
Malignant ectomesenchymoma of the nasal cavity. ( 22563163 )
2011
10
Malignant ectomesenchymoma of the nasal cavity. ( 17395109 )
2007
11
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. ( 18043289 )
2007
12
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. ( 15747131 )
2005
13
A neonate with malignant ectomesenchymoma. ( 16363352 )
2005
14
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. ( 15735865 )
2005
15
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. ( 12469232 )
2003
16
Malignant ectomesenchymoma of the nose. Case report and review of the literature. ( 12624508 )
2003
17
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. ( 11787870 )
2002
18
Primary malignant ectomesenchymoma of the kidney. ( 12383226 )
2002
19
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. ( 12161212 )
2002
20
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. ( 11297080 )
2001
21
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. ( 10503746 )
1999
22
Malignant ectomesenchymoma. Case report and review of the literature. ( 10494059 )
1999
23
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. ( 9544170 )
1998
24
Primary malignant ectomesenchymoma of the orbit. ( 9065587 )
1997
25
Malignant ectomesenchymoma in childhood. ( 9025858 )
1996
26
Primary malignant ectomesenchymoma of the orbit. ( 1627516 )
1992
27
Parapharyngeal malignant ectomesenchymoma: combined malignant fibrous histiocytoma and primitive neuroectodermal tumour with neuroglial differentiation. ( 3035038 )
1987
28
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. ( 2950992 )
1987
29
Malignant ectomesenchymoma (neoplasm of mixed mesenchymal and neuroepithelial origin) of wrist joint. ( 6491565 )
1984

Variations for Malignant Ectomesenchymoma

Expression for Malignant Ectomesenchymoma

Search GEO for disease gene expression data for Malignant Ectomesenchymoma.

Pathways for Malignant Ectomesenchymoma

Pathways related to Malignant Ectomesenchymoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.28 HMGA1 HRAS
2 10.1 FOXO1 HRAS

GO Terms for Malignant Ectomesenchymoma

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

# Name GO ID Score Top Affiliating Genes
1 senescence-associated heterochromatin focus GO:0035985 8.62 HMGA1 HMGA2

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.67 FOXO1 HMGA1 HMGA2 HRAS
2 fat cell differentiation GO:0045444 9.4 FOXO1 HMGA2
3 base-excision repair GO:0006284 9.32 HMGA1 HMGA2
4 regulation of heart contraction GO:0008016 9.26 DES S100A1
5 positive regulation of cellular senescence GO:2000774 9.16 HMGA1 HMGA2
6 senescence-associated heterochromatin focus assembly GO:0035986 8.96 HMGA1 HMGA2
7 oncogene-induced cell senescence GO:0090402 8.62 HMGA1 HMGA2

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

# Name GO ID Score Top Affiliating Genes
1 transcription factor binding GO:0008134 9.33 FOXO1 HMGA1 HMGA2
2 DNA-(apurinic or apyrimidinic site) endonuclease activity GO:0003906 8.96 HMGA1 HMGA2
3 AT DNA binding GO:0003680 8.62 HMGA1 HMGA2

Sources for Malignant Ectomesenchymoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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