MCID: MLG133
MIFTS: 33

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 rhabdomyosarcoma. An important gene associated with Malignant Ectomesenchymoma is HMGA1 (High Mobility Group AT-Hook 1), and among its related pathways/superpathways are Telomere Extension by Telomerase and PI3K-AKT-mTOR signaling pathway and therapeutic opportunities. The drugs Mechlorethamine and Irinotecan have been mentioned in the context of this disorder. Affiliated tissues include lung, kidney and skin.

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 66)
# Related Disease Score Top Affiliating Genes
1 ectomesenchymoma 30.8 DES ENO2 FOXO1 HMGA1 HMGA2 HRAS
2 rhabdomyosarcoma 30.2 DES ENO2 FOXO1
3 embryonal rhabdomyosarcoma 29.8 DES FOXO1 HRAS
4 spindle cell lipoma 10.2 DES FOXO1
5 exophthalmos 10.2
6 primitive neuroectodermal tumor of the cervix uteri 10.2 DES ENO2
7 epulis 10.2 DES ENO2
8 ossifying fibromyxoid tumor 10.2 DES ENO2
9 epithelioid leiomyosarcoma 10.2 DES ENO2
10 desmoplastic small round cell tumor 10.1 DES ENO2
11 ischemic fasciitis 10.1 DES S100A1
12 reticulum cell sarcoma 10.1 DES S100A1
13 extraskeletal ewing sarcoma 10.1 DES ENO2
14 glomus tumor 10.1 DES ENO2
15 granular cell tumor 10.1 DES ENO2
16 chordoma 10.1 DES S100A1
17 papillary tumor of the pineal region 10.1 ENO2 S100A1
18 congenital epulis 10.1 DES ENO2
19 medullomyoblastoma 10.1 DES ENO2
20 chondromyxoid fibroma 10.1 DES S100A1
21 chondroid chordoma 10.1 ENO2 S100A1
22 meningeal melanomatosis 10.0 ENO2 HRAS
23 neurilemmoma 10.0 DES ENO2
24 milia, multiple eruptive 10.0
25 sarcoma 10.0
26 malignant fibroxanthoma 10.0
27 histiocytoma 10.0
28 spindle cell sarcoma 10.0
29 fibrous histiocytoma 10.0
30 undifferentiated pleomorphic sarcoma 10.0
31 ring chromosome 1 10.0 HMGA1 HMGA2
32 pelvic lipomatosis 10.0 HMGA1 HMGA2
33 pericardial mesothelioma 10.0 ENO2 S100A1
34 mesenchymoma 10.0 DES HMGA2
35 cavernous hemangioma 10.0 DES S100A1
36 duodenum cancer 10.0 ENO2 HRAS
37 mixed cell type cancer 10.0 ENO2 HRAS
38 alveolar soft part sarcoma 10.0 DES ENO2
39 medulloblastoma 10.0
40 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.0
41 atypical teratoid rhabdoid tumor 10.0
42 gangliocytoma 10.0
43 rhabdoid cancer 10.0
44 ganglioneuroma 10.0
45 liver angiosarcoma 10.0 ENO2 HRAS
46 dedifferentiated liposarcoma 10.0 DES HMGA2
47 adenosquamous lung carcinoma 10.0 ENO2 HRAS
48 liposarcoma 10.0 DES HMGA2
49 smooth muscle tumor 9.9 DES HMGA2
50 bile duct adenocarcinoma 9.9 HRAS 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 29)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
2
Irinotecan Approved, Investigational Phase 3 100286-90-6, 97682-44-5 60838
3
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Ifosfamide Approved Phase 3 3778-73-2 3690
6
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
7
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
8
Mesna Approved, Investigational Phase 3 3375-50-6 598
9
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
10
Lenograstim Approved, Investigational Phase 3 135968-09-1
11
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
12
Camptothecin Experimental Phase 3 7689-03-4
13
Doxil Approved June 1999 Phase 3 31703
14 topoisomerase I inhibitors Phase 3
15 Antimitotic Agents Phase 3
16
Isophosphamide mustard Phase 3 0
17 Antineoplastic Agents, Alkylating Phase 3
18 Immunosuppressive Agents Phase 3
19 Etoposide phosphate Phase 3
20 Anti-Infective Agents Phase 3
21 Antineoplastic Agents, Phytogenic Phase 3
22 Antirheumatic Agents Phase 3
23 Anti-Bacterial Agents Phase 3
24 Adjuvants, Immunologic Phase 3
25 Antibiotics, Antitubercular Phase 3
26 Alkylating Agents Phase 3
27 Nucleic Acid Synthesis Inhibitors Phase 3
28 Topoisomerase Inhibitors Phase 3
29 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 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
Lung, Kidney, Skin, Liver, Heart, Cervix, Smooth Muscle

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 )
2018
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 in the wrist of a child: case report and review of the literature. ( 15735865 )
2005
14
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. ( 15747131 )
2005
15
A neonate with malignant ectomesenchymoma. ( 16363352 )
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
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. ( 12161212 )
2002
20
Primary malignant ectomesenchymoma of the kidney. ( 12383226 )
2002
21
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. ( 11297080 )
2001
22
Malignant ectomesenchymoma. Case report and review of the literature. ( 10494059 )
1999
23
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. ( 10503746 )
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
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. ( 2950992 )
1987
29
Parapharyngeal malignant ectomesenchymoma: combined malignant fibrous histiocytoma and primitive neuroectodermal tumour with neuroglial differentiation. ( 3035038 )
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
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
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45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
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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