MCID: MLG133
MIFTS: 32

Malignant Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Malignant Ectomesenchymoma

MalaCards integrated aliases for Malignant Ectomesenchymoma:

Name: Malignant Ectomesenchymoma 54
Rhabdomyosarcoma with Ganglionic Differentiation 74
Ectomesenchymoma 54

Classifications:



External Ids:

UMLS 74 C0431111

Summaries for Malignant Ectomesenchymoma

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

Wikipedia : 77 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 30.8 DES ENO2 FOXO1 HMGA1 HMGA2 HRAS
2 embryonal rhabdomyosarcoma 29.8 DES FOXO1 HRAS
3 rhabdomyosarcoma 29.7 DES ENO2 FOXO1
4 ring chromosome 1 10.2 HMGA1 HMGA2
5 pelvic lipomatosis 10.2 HMGA1 HMGA2
6 epithelial-myoepithelial carcinoma 10.2 HMGA2 HRAS
7 mesenchymoma 10.1 DES HMGA2
8 bile duct adenocarcinoma 10.1 HRAS S100A1
9 dedifferentiated liposarcoma 10.0 DES HMGA2
10 liposarcoma 10.0 DES HMGA2
11 spindle cell lipoma 10.0 DES FOXO1
12 sarcoma 10.0
13 malignant fibroxanthoma 10.0
14 histiocytoma 10.0
15 spindle cell sarcoma 10.0
16 fibrous histiocytoma 10.0
17 smooth muscle tumor 10.0 DES HMGA2
18 ischemic fasciitis 10.0 DES S100A1
19 reticulum cell sarcoma 10.0 DES S100A1
20 cell type benign neoplasm 10.0 HMGA2 HRAS
21 chordoma 10.0 DES S100A1
22 medulloblastoma 10.0
23 atypical teratoid rhabdoid tumor 10.0
24 rhabdoid cancer 10.0
25 ganglioneuroma 10.0
26 meningeal melanomatosis 10.0 ENO2 HRAS
27 duodenum cancer 10.0 ENO2 HRAS
28 mixed cell type cancer 9.9 ENO2 HRAS
29 angiomyoma 9.9 ENO2 HMGA2
30 liver angiosarcoma 9.9 ENO2 HRAS
31 chondromyxoid fibroma 9.9 DES S100A1
32 adenosquamous lung carcinoma 9.9 ENO2 HRAS
33 papillary tumor of the pineal region 9.9 ENO2 S100A1
34 central nervous system melanocytic neoplasm 9.9 ENO2 HRAS
35 skin benign neoplasm 9.9 ENO2 HRAS
36 chondroid chordoma 9.9 ENO2 S100A1
37 lung benign neoplasm 9.9 ENO2 HRAS
38 carcinosarcoma 9.9 DES HRAS
39 cavernous hemangioma 9.9 DES S100A1
40 primitive neuroectodermal tumor of the cervix uteri 9.9 DES ENO2
41 epulis 9.9 DES ENO2
42 ossifying fibromyxoid tumor 9.9 DES ENO2
43 epithelioid leiomyosarcoma 9.9 DES ENO2
44 lipomatosis, multiple 9.8 DES HMGA1 HMGA2
45 desmoplastic small round cell tumor 9.8 DES ENO2
46 extraskeletal ewing sarcoma 9.8 DES ENO2
47 pericardial mesothelioma 9.8 ENO2 S100A1
48 glomus tumor 9.8 DES ENO2
49 granular cell tumor 9.8 DES ENO2
50 congenital epulis 9.8 DES ENO2

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 29)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mesna Approved, Investigational Phase 3 3375-50-6 598
2
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
3
Ifosfamide Approved Phase 3 3778-73-2 3690
4
Lenograstim Approved, Investigational Phase 3 135968-09-1
5
Cyclophosphamide Approved, Investigational Phase 3 6055-19-2, 50-18-0 2907
6
Etoposide Approved Phase 3 33419-42-0 36462
7
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
8
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
9
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
10
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
11
Irinotecan Approved, Investigational Phase 3 100286-90-6, 97682-44-5 60838
12
Doxil Approved June 1999 Phase 3 31703
13
Camptothecin Experimental Phase 3 7689-03-4
14
Isophosphamide mustard Phase 3 0
15 Anti-Bacterial Agents Phase 3
16 Antineoplastic Agents, Alkylating Phase 3
17 Etoposide phosphate Phase 3
18 Anti-Infective Agents Phase 3
19 Adjuvants, Immunologic Phase 3
20 Immunosuppressive Agents Phase 3
21 Immunologic Factors Phase 3
22 Nucleic Acid Synthesis Inhibitors Phase 3
23 Topoisomerase Inhibitors Phase 3
24 Alkylating Agents Phase 3
25 Antibiotics, Antitubercular Phase 3
26 Antimitotic Agents Phase 3
27 topoisomerase I inhibitors Phase 3
28 Antineoplastic Agents, Phytogenic Phase 3
29 Antirheumatic Agents 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 Completed 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:

42
Kidney, Lung

Publications for Malignant Ectomesenchymoma

Articles related to Malignant Ectomesenchymoma:

(show all 30)
# Title Authors Year
1
Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature. ( 30907031 )
2019
2
Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor. ( 28994342 )
2017
3
Malignant Ectomesenchymoma: A Potential Pitfall of Diagnosis in the Spectrum of Pediatric Small Blue Round Cell Tumors. ( 28968263 )
2017
4
Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma. ( 26872011 )
2016
5
An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis. ( 26793539 )
2015
6
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. ( 25405050 )
2014
7
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). ( 22535600 )
2013
8
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. ( 23174743 )
2012
9
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. ( 22026142 )
2011
10
Malignant ectomesenchymoma of the nasal cavity. ( 22563163 )
2011
11
Malignant ectomesenchymoma of the nasal cavity. ( 17395109 )
2007
12
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. ( 18043289 )
2007
13
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. ( 15747131 )
2005
14
A neonate with malignant ectomesenchymoma. ( 16363352 )
2005
15
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. ( 15735865 )
2005
16
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. ( 12469232 )
2003
17
Malignant ectomesenchymoma of the nose. Case report and review of the literature. ( 12624508 )
2003
18
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. ( 11787870 )
2002
19
Primary malignant ectomesenchymoma of the kidney. ( 12383226 )
2002
20
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. ( 12161212 )
2002
21
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. ( 11297080 )
2001
22
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. ( 10503746 )
1999
23
Malignant ectomesenchymoma. Case report and review of the literature. ( 10494059 )
1999
24
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. ( 9544170 )
1998
25
Primary malignant ectomesenchymoma of the orbit. ( 9065587 )
1997
26
Malignant ectomesenchymoma in childhood. ( 9025858 )
1996
27
Primary malignant ectomesenchymoma of the orbit. ( 1627516 )
1992
28
Parapharyngeal malignant ectomesenchymoma: combined malignant fibrous histiocytoma and primitive neuroectodermal tumour with neuroglial differentiation. ( 3035038 )
1987
29
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. ( 2950992 )
1987
30
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
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9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
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31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
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50 NCI
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55 NINDS
56 Novoseek
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59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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