MCID: ECT002
MIFTS: 35

Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Ectomesenchymoma

MalaCards integrated aliases for Ectomesenchymoma:

Name: Ectomesenchymoma 12 6 15
Rhabdomyosarcoma with Ganglionic Differentiation 12 74

Classifications:



External Ids:

Disease Ontology 12 DOID:4055
NCIt 51 C4716
UMLS 74 C0431111

Summaries for Ectomesenchymoma

MalaCards based summary : Ectomesenchymoma, also known as rhabdomyosarcoma with ganglionic differentiation, is related to malignant ectomesenchymoma and rhabdomyosarcoma. An important gene associated with Ectomesenchymoma is FOXO1 (Forkhead Box O1), and among its related pathways/superpathways are Thyroid hormone signaling pathway and Glioblastoma Multiforme. The drugs Mechlorethamine and Irinotecan have been mentioned in the context of this disorder. Affiliated tissues include lung, tongue and kidney.

Wikipedia : 77 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 57)
# Related Disease Score Top Affiliating Genes
1 malignant ectomesenchymoma 32.0 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 10.1 DES FOXO1
5 mesenchymoma 10.1 DES HMGA2
6 exophthalmos 10.1
7 ring chromosome 1 10.1 HMGA1 HMGA2
8 pelvic lipomatosis 10.1 HMGA1 HMGA2
9 dedifferentiated liposarcoma 10.1 DES HMGA2
10 liposarcoma 10.1 DES HMGA2
11 smooth muscle tumor 10.1 DES HMGA2
12 angiomyoma 10.1 ENO2 HMGA2
13 primitive neuroectodermal tumor of the cervix uteri 10.0 DES ENO2
14 epulis 10.0 DES ENO2
15 ossifying fibromyxoid tumor 10.0 DES ENO2
16 epithelioid leiomyosarcoma 10.0 DES ENO2
17 leiomyosarcoma 10.0 DES ENO2
18 desmoplastic small round cell tumor 10.0 DES ENO2
19 extraskeletal ewing sarcoma 10.0 DES ENO2
20 glomus tumor 10.0 DES ENO2
21 granular cell tumor 10.0 DES ENO2
22 medulloblastoma 10.0
23 milia, multiple eruptive 10.0
24 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.0
25 sarcoma 10.0
26 malignant fibroxanthoma 10.0
27 atypical teratoid rhabdoid tumor 10.0
28 gangliocytoma 10.0
29 rhabdoid cancer 10.0
30 histiocytoma 10.0
31 spindle cell sarcoma 10.0
32 fibrous histiocytoma 10.0
33 ganglioneuroma 10.0
34 undifferentiated pleomorphic sarcoma 10.0
35 congenital epulis 10.0 DES ENO2
36 medullomyoblastoma 10.0 DES ENO2
37 sarcoma, synovial 9.9 DES HMGA2
38 epithelial-myoepithelial carcinoma 9.9 HMGA2 HRAS
39 neurilemmoma 9.9 DES ENO2
40 alveolar soft part sarcoma 9.9 DES ENO2
41 meningeal melanomatosis 9.8 ENO2 HRAS
42 lipomatosis, multiple 9.8 DES HMGA1 HMGA2
43 leiomyoma 9.8 DES HMGA2
44 duodenum cancer 9.8 ENO2 HRAS
45 muscle cancer 9.8 DES ENO2 FOXO1
46 cell type benign neoplasm 9.8 HMGA2 HRAS
47 mixed cell type cancer 9.8 ENO2 HRAS
48 rhabdomyosarcoma 2 9.8 DES ENO2 FOXO1
49 liver angiosarcoma 9.8 ENO2 HRAS
50 adenosquamous lung carcinoma 9.8 ENO2 HRAS

Graphical network of the top 20 diseases related to Ectomesenchymoma:



Diseases related to Ectomesenchymoma

Symptoms & Phenotypes for Ectomesenchymoma

Drugs & Therapeutics for Ectomesenchymoma

Drugs for 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 Ectomesenchymoma

Genetic Tests for Ectomesenchymoma

Anatomical Context for Ectomesenchymoma

MalaCards organs/tissues related to Ectomesenchymoma:

42
Lung, Tongue, Kidney, Prostate, Medulla Oblongata, Heart, Smooth Muscle

Publications for Ectomesenchymoma

Articles related to Ectomesenchymoma:

(show all 45)
# 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
A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum. ( 26339431 )
2015
6
An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis. ( 26793539 )
2015
7
Ectomesenchymoma with embryonal rhabdomyosarcoma and ganglioneuroma, arising in association with benign triton tumor of the tongue. ( 24650353 )
2014
8
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. ( 25405050 )
2014
9
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). ( 22535600 )
2013
10
Ectomesenchymoma with t(1;12)(p32;p13) evolving from embryonal rhabdomyosarcoma shows no rearrangement of ETV6. ( 21803398 )
2012
11
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. ( 23174743 )
2012
12
A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri. ( 21194292 )
2011
13
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. ( 22026142 )
2011
14
Malignant ectomesenchymoma of the nasal cavity. ( 22563163 )
2011
15
Retroperitoneal ectomesenchymoma: a rare childhood tumor. ( 20090275 )
2010
16
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
17
Malignant ectomesenchymoma of the nasal cavity. ( 17395109 )
2007
18
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. ( 18043289 )
2007
19
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. ( 15735865 )
2005
20
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. ( 15747131 )
2005
21
A neonate with malignant ectomesenchymoma. ( 16363352 )
2005
22
Ectomesenchymoma: case report and review of the literature. ( 15074381 )
2004
23
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. ( 12469232 )
2003
24
Malignant ectomesenchymoma of the nose. Case report and review of the literature. ( 12624508 )
2003
25
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. ( 11787870 )
2002
26
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. ( 12161212 )
2002
27
Primary malignant ectomesenchymoma of the kidney. ( 12383226 )
2002
28
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. ( 11297080 )
2001
29
Ectomesenchymoma of the prostate: histological diagnostic criteria. ( 9914362 )
1999
30
Malignant ectomesenchymoma. Case report and review of the literature. ( 10494059 )
1999
31
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. ( 10503746 )
1999
32
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. ( 9544170 )
1998
33
Primary malignant ectomesenchymoma of the orbit. ( 9065587 )
1997
34
Ectomesenchymoma: one or two tumors? Case report and review of the literature. ( 9314261 )
1997
35
Ectomesenchymoma of the medulla oblongata in a calf. ( 9421949 )
1997
36
Malignant ectomesenchymoma in childhood. ( 9025858 )
1996
37
Ectomesenchymal hamartoma (benign "ectomesenchymoma") of the VIIIth nerve: case report. ( 8584163 )
1995
38
Primary malignant ectomesenchymoma of the orbit. ( 1627516 )
1992
39
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. ( 2950992 )
1987
40
Parapharyngeal malignant ectomesenchymoma: combined malignant fibrous histiocytoma and primitive neuroectodermal tumour with neuroglial differentiation. ( 3035038 )
1987
41
Case of the month: ectomesenchymoma (malignant testicular tumor). ( 6142120 )
1984
42
Malignant ectomesenchymoma (neoplasm of mixed mesenchymal and neuroepithelial origin) of wrist joint. ( 6491565 )
1984
43
Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh. ( 6492817 )
1984
44
Ectomesenchymoma. Report of two cases. ( 6819710 )
1982
45
Ectomesenchymoma. A malignant tumor of migratory neural crest (ectomesenchyme) remnants showing ganglionic, schwannian, melanocytic and rhabdomyoblastic differentiation. ( 872048 )
1977

Variations for Ectomesenchymoma

ClinVar genetic disease variations for Ectomesenchymoma:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 MSH2 NM_000251.2(MSH2): c.2732T> G (p.Leu911Arg) single nucleotide variant Uncertain significance rs41295182 GRCh37 Chromosome 2, 47710015: 47710015
2 MSH2 NM_000251.2(MSH2): c.2732T> G (p.Leu911Arg) single nucleotide variant Uncertain significance rs41295182 GRCh38 Chromosome 2, 47482876: 47482876

Expression for Ectomesenchymoma

Search GEO for disease gene expression data for Ectomesenchymoma.

Pathways for Ectomesenchymoma

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, DNA-templated GO:0045893 9.63 FOXO1 HMGA1 HMGA2
2 positive regulation of transcription by RNA polymerase II GO:0045944 9.62 FOXO1 HMGA1 HMGA2 HRAS
3 negative regulation of transcription, DNA-templated GO:0045892 9.58 FOXO1 HMGA1 HMGA2
4 response to virus GO:0009615 9.43 HMGA1 HMGA2
5 fat cell differentiation GO:0045444 9.37 FOXO1 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 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 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
39 LifeMap
41 LOVD
43 MedGen
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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