MCID: ECT002
MIFTS: 35

Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Ectomesenchymoma

MalaCards integrated aliases for Ectomesenchymoma:

Name: Ectomesenchymoma 12 15
Rhabdomyosarcoma with Ganglionic Differentiation 12 73

Classifications:



External Ids:

Disease Ontology 12 DOID:4055
NCIt 50 C4716
SNOMED-CT 68 128750008
UMLS 73 C0431111

Summaries for Ectomesenchymoma

MalaCards based summary : Ectomesenchymoma, also known as rhabdomyosarcoma with ganglionic differentiation, is related to malignant ectomesenchymoma and atypical teratoid rhabdoid tumor. An important gene associated with Ectomesenchymoma is HMGA2 (High Mobility Group AT-Hook 2), and among its related pathways/superpathways are Immune response IL-2 activation and signaling pathway and Senescence and Autophagy in Cancer. The drugs Cyclophosphamide and Dactinomycin have been mentioned in the context of this disorder. Affiliated tissues include kidney, tongue and medulla oblongata, and related phenotypes are Increased Nanog expression and Increased Nanog expression

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

Related Diseases for Ectomesenchymoma

Diseases in the Ectomesenchymoma family:

Malignant Ectomesenchymoma

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

(show top 50) (show all 64)
# Related Disease Score Top Affiliating Genes
1 malignant ectomesenchymoma 30.8 DES ENO2 HMGA1 HMGA2 HRAS
2 atypical teratoid rhabdoid tumor 29.8 DES HMGA2
3 rhabdomyosarcoma 29.5 DES ENO2 PAX3
4 embryonal rhabdomyosarcoma 29.3 DES HRAS PAX3
5 medulloblastoma 28.7 DES ENO2 PAX3
6 angiomyoma 10.4 ENO2 HMGA2
7 epithelial-myoepithelial carcinoma 10.3 HMGA2 HRAS
8 meningeal melanomatosis 10.3 ENO2 HRAS
9 mixed cell type cancer 10.3 ENO2 HRAS
10 gastrointestinal system benign neoplasm 10.3 HMGA2 HRAS
11 adenosquamous cell lung carcinoma 10.2 ENO2 HRAS
12 mesenchymoma 10.2 DES HMGA2
13 duodenum cancer 10.2 ENO2 HRAS
14 liver angiosarcoma 10.2 ENO2 HRAS
15 central nervous system melanocytic neoplasm 10.2 ENO2 HRAS
16 dedifferentiated liposarcoma 10.2 DES HMGA2
17 breast rhabdomyosarcoma 10.2 DES ENO2
18 primitive neuroectodermal tumor of the cervix uteri 10.2 DES ENO2
19 epulis 10.2 DES ENO2
20 ossifying fibromyxoid tumor 10.2 DES ENO2
21 epithelioid leiomyosarcoma 10.2 DES ENO2
22 skin benign neoplasm 10.2 ENO2 HRAS
23 smooth muscle tumor 10.2 DES HMGA2
24 cell type benign neoplasm 10.2 HMGA2 HRAS
25 lung benign neoplasm 10.2 ENO2 HRAS
26 desmoplastic small round cell tumor 10.2 DES ENO2
27 extraskeletal ewing sarcoma 10.1 DES ENO2
28 glomus tumor 10.1 DES ENO2
29 integumentary system cancer 10.1 ENO2 HRAS
30 granular cell tumor 10.1 DES ENO2
31 congenital epulis 10.1 DES ENO2
32 dyschromatosis symmetrica hereditaria 10.1 HRAS PAX3
33 botryoid rhabdomyosarcoma 10.1 DES PAX3
34 medullomyoblastoma 10.1 DES ENO2
35 malignant fibrous histiocytoma of bone 10.1 DES HRAS
36 glomangioma 10.1 DES ENO2
37 spindle cell carcinoma 10.1 DES ENO2
38 neurilemmoma 10.0 DES ENO2
39 ring chromosome 1 10.0 HMGA1 HMGA2
40 pelvic lipomatosis 10.0 HMGA1 HMGA2
41 sarcoma, synovial 10.0 DES HMGA2
42 respiratory system cancer 10.0 ENO2 HRAS
43 alveolar soft part sarcoma 10.0 DES ENO2
44 endocrine gland cancer 10.0 ENO2 HRAS
45 liposarcoma 10.0 DES HMGA2
46 sinusitis 9.9
47 leiomyosarcoma 9.9 DES ENO2
48 cell type cancer 9.9 ENO2 HRAS
49 sarcoma 9.8
50 prostatitis 9.8

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

Drugs & Therapeutics for Ectomesenchymoma

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

(show all 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
2
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
3
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Ifosfamide Approved Phase 3 3778-73-2 3690
6
Irinotecan Approved, Investigational Phase 3 97682-44-5, 100286-90-6 60838
7
Lenograstim Approved, Investigational Phase 3 135968-09-1
8
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
9
Mesna Approved, Investigational Phase 3 3375-50-6 598
10
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
11
Camptothecin Experimental Phase 3 7689-03-4
12
Doxil Approved June 1999 Phase 3 31703
13 Adjuvants, Immunologic Phase 3
14 Alkylating Agents Phase 3
15 Anti-Bacterial Agents Phase 3
16 Antibiotics, Antitubercular Phase 3
17 Anti-Infective Agents Phase 3
18 Antimitotic Agents Phase 3
19 Antineoplastic Agents, Alkylating Phase 3
20 Antineoplastic Agents, Phytogenic Phase 3
21 Antirheumatic Agents Phase 3
22 Etoposide phosphate Phase 3
23 Immunosuppressive Agents Phase 3
24
Isophosphamide mustard Phase 3 0
25 Nucleic Acid Synthesis Inhibitors Phase 3
26 topoisomerase I inhibitors Phase 3
27 Topoisomerase Inhibitors 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 Active, not recruiting NCT00354744 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;irinotecan hydrochloride;vincristine sulfate

Search NIH Clinical Center for Ectomesenchymoma

Genetic Tests for Ectomesenchymoma

Anatomical Context for Ectomesenchymoma

MalaCards organs/tissues related to Ectomesenchymoma:

41
Kidney, Tongue, Medulla Oblongata, Prostate, Lung

Publications for Ectomesenchymoma

Articles related to Ectomesenchymoma:

(show all 43)
# 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
A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum. ( 26339431 )
2015
6
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. ( 25405050 )
2014
7
Ectomesenchymoma with embryonal rhabdomyosarcoma and ganglioneuroma, arising in association with benign triton tumor of the tongue. ( 24650353 )
2014
8
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). ( 22535600 )
2013
9
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. ( 23174743 )
2012
10
Ectomesenchymoma with t(1;12)(p32;p13) evolving from embryonal rhabdomyosarcoma shows no rearrangement of ETV6. ( 21803398 )
2012
11
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. ( 22026142 )
2011
12
A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri. ( 21194292 )
2011
13
Malignant ectomesenchymoma of the nasal cavity. ( 22563163 )
2011
14
Retroperitoneal ectomesenchymoma: a rare childhood tumor. ( 20090275 )
2010
15
Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor. ( 17431644 )
2007
16
Malignant ectomesenchymoma of the nasal cavity. ( 17395109 )
2007
17
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. ( 18043289 )
2007
18
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. ( 15747131 )
2005
19
A neonate with malignant ectomesenchymoma. ( 16363352 )
2005
20
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. ( 15735865 )
2005
21
Ectomesenchymoma: case report and review of the literature. ( 15074381 )
2004
22
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. ( 12469232 )
2003
23
Malignant ectomesenchymoma of the nose. Case report and review of the literature. ( 12624508 )
2003
24
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. ( 11787870 )
2002
25
Primary malignant ectomesenchymoma of the kidney. ( 12383226 )
2002
26
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. ( 12161212 )
2002
27
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. ( 11297080 )
2001
28
Ectomesenchymoma of the prostate: histological diagnostic criteria. ( 9914362 )
1999
29
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. ( 10503746 )
1999
30
Malignant ectomesenchymoma. Case report and review of the literature. ( 10494059 )
1999
31
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. ( 9544170 )
1998
32
Primary malignant ectomesenchymoma of the orbit. ( 9065587 )
1997
33
Ectomesenchymoma: one or two tumors? Case report and review of the literature. ( 9314261 )
1997
34
Ectomesenchymoma of the medulla oblongata in a calf. ( 9421949 )
1997
35
Malignant ectomesenchymoma in childhood. ( 9025858 )
1996
36
Primary malignant ectomesenchymoma of the orbit. ( 1627516 )
1992
37
Parapharyngeal malignant ectomesenchymoma: combined malignant fibrous histiocytoma and primitive neuroectodermal tumour with neuroglial differentiation. ( 3035038 )
1987
38
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. ( 2950992 )
1987
39
Malignant ectomesenchymoma (neoplasm of mixed mesenchymal and neuroepithelial origin) of wrist joint. ( 6491565 )
1984
40
Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh. ( 6492817 )
1984
41
Case of the month: ectomesenchymoma (malignant testicular tumor). ( 6142120 )
1984
42
Ectomesenchymoma. Report of two cases. ( 6819710 )
1982
43
Ectomesenchymoma. A malignant tumor of migratory neural crest (ectomesenchyme) remnants showing ganglionic, schwannian, melanocytic and rhabdomyoblastic differentiation. ( 872048 )
1977

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
Show member pathways
10.98 HMGA1 HRAS
2 10.65 HMGA1 HRAS

GO Terms for Ectomesenchymoma

Cellular components related to 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 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 9.62 HMGA1 HMGA2 HRAS PAX3
2 transcription by RNA polymerase II GO:0006366 9.61 HMGA1 HMGA2 PAX3
3 positive regulation of transcription, DNA-templated GO:0045893 9.58 HMGA1 HMGA2 PAX3
4 animal organ morphogenesis GO:0009887 9.46 HRAS PAX3
5 response to virus GO:0009615 9.37 HMGA1 HMGA2
6 base-excision repair GO:0006284 9.26 HMGA1 HMGA2
7 positive regulation of cellular senescence GO:2000774 9.16 HMGA1 HMGA2
8 senescence-associated heterochromatin focus assembly GO:0035986 8.96 HMGA1 HMGA2
9 oncogene-induced cell senescence GO:0090402 8.62 HMGA1 HMGA2

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 8.96 HMGA1 HMGA2
2 AT DNA binding GO:0003680 8.62 HMGA1 HMGA2

Sources for 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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