MCID: ECT002
MIFTS: 33

Ectomesenchymoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Ectomesenchymoma

MalaCards integrated aliases for Ectomesenchymoma:

Name: Ectomesenchymoma 12 52 6 15
Rhabdomyosarcoma with Ganglionic Differentiation 12 71
Malignant Ectomesenchymoma 52

Classifications:



External Ids:

Disease Ontology 12 DOID:4055
NCIt 49 C4716
UMLS 71 C0431111

Summaries for Ectomesenchymoma

MalaCards based summary : Ectomesenchymoma, also known as rhabdomyosarcoma with ganglionic differentiation, is related to neurilemmoma and embryonal sarcoma. An important gene associated with Ectomesenchymoma is FOXO1 (Forkhead Box O1), and among its related pathways/superpathways is PI3K-AKT-mTOR signaling pathway and therapeutic opportunities. The drugs Irinotecan and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include bone, prostate and kidney.

Wikipedia : 74 Ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that occurs mainly... more...

Related Diseases for Ectomesenchymoma

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

(show top 50) (show all 160)
# Related Disease Score Top Affiliating Genes
1 neurilemmoma 30.2 S100A1 ENO2 DES
2 embryonal sarcoma 30.2 MYOG DES
3 embryonal rhabdomyosarcoma 30.1 MYOG HRAS FOXO1 DES
4 rhabdoid cancer 30.1 MYOG ENO2
5 malignant fibroxanthoma 30.1 MYOG HMGA2 DES
6 spindle cell sarcoma 30.0 MYOG DES
7 mesenchymoma 29.9 MYOG HMGA2 DES
8 malignant peripheral nerve sheath tumor 29.8 S100A1 MYOG HMGA2
9 atypical teratoid rhabdoid tumor 29.7 S100A1 HMGA2 DES
10 liposarcoma 29.5 MYOG MSH2 HMGA2 DES
11 rhabdomyosarcoma 2 28.8 MYOG FOXO1 ENO2 DES CD99
12 leiomyosarcoma 28.8 S100A1 MYOG HMGA2 ENO2 DES
13 rhabdomyosarcoma 28.7 MYOG MSH2 HRAS FOXO1 ENO2 DES
14 ewing sarcoma 28.6 MYOG FOXO1 ENO2 DES CD99
15 medulloblastoma 28.6 MYOG MSH2 HRAS FOXO1 ENO2 DES
16 gangliocytoma 10.4
17 ganglioneuroma 10.4
18 neuroblastoma 10.4
19 rare tumor 10.4
20 appendix carcinoid tumor 10.4 MSH2 ENO2
21 cervical adenoma malignum 10.4 MSH2 HMGA2
22 cervical mucinous adenocarcinoma 10.3 MSH2 HMGA2
23 primitive neuroectodermal tumor of the cervix uteri 10.3 ENO2 DES
24 congenital epulis 10.3 ENO2 DES
25 epulis 10.3 ENO2 DES
26 spindle cell thymoma 10.3 ENO2 DES
27 ring chromosome 1 10.3 HMGA2 HMGA1
28 ring chromosome 10.3 HMGA2 HMGA1
29 ganglioneuroblastoma 10.2
30 rare soft tissue tumor 10.2
31 myh-associated polyposis 10.2 MSH2 HRAS
32 epithelioid leiomyosarcoma 10.2 ENO2 DES
33 ascending colon cancer 10.2 MSH2 HRAS
34 prostate embryonal rhabdomyosarcoma 10.2 MYOG FOXO1
35 duodenal benign neoplasm 10.2 HRAS ENO2
36 ampulla of vater neoplasm 10.2 HRAS ENO2
37 small intestine adenocarcinoma 10.2 MSH2 HRAS
38 central nervous system melanocytic neoplasm 10.2 HRAS ENO2
39 myoblastoma 10.2 MYOG ENO2
40 duodenum adenocarcinoma 10.2 MSH2 HRAS
41 parameningeal embryonal rhabdomyosarcoma 10.2 MYOG FOXO1
42 endometrioid ovary carcinoma 10.2 MSH2 HRAS
43 endobronchial leiomyoma 10.2 MYOG ENO2
44 vagina sarcoma 10.2 MYOG ENO2
45 melanocytic nevus syndrome, congenital 10.2 MSH2 HRAS
46 inflammatory leiomyosarcoma 10.2 MYOG DES
47 gallbladder sarcoma 10.2 MYOG DES
48 chondrosarcoma 10.2
49 astrocytoma 10.2
50 histiocytoma 10.2

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 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Irinotecan Approved, Investigational Phase 3 97682-44-5, 100286-90-6 60838
2
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
3
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
6
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
7
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
8
Ifosfamide Approved Phase 3 3778-73-2 3690
9
Lenograstim Approved, Investigational Phase 3 135968-09-1
10
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
11
Mesna Approved, Investigational Phase 3 3375-50-6 598
12
Camptothecin Experimental Phase 3 7689-03-4
13 Alkylating Agents Phase 3
14 topoisomerase I inhibitors Phase 3
15
Isophosphamide mustard Phase 3 0
16 Anti-Infective Agents Phase 3
17 Topoisomerase Inhibitors Phase 3
18 Antibiotics, Antitubercular Phase 3
19 Anti-Bacterial Agents Phase 3
20 Adjuvants, Immunologic Phase 3
21
Liposomal doxorubicin Phase 3 31703
22 Immunosuppressive Agents Phase 3
23 Antirheumatic Agents Phase 3
24 Etoposide phosphate Phase 3
25 Immunologic Factors Phase 3
26 Antimitotic Agents Phase 3
27
Tirapazamine Investigational Phase 2 27314-97-2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Intensive Multi-Agent Therapy, Including Dose-Compressed Cycles of Ifosfamide/Etoposide (IE) and Vincristine/Doxorubicin/Cyclophosphamide (VDC) for Patients With High-Risk Rhabdomyosarcoma Completed NCT00354744 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;irinotecan hydrochloride;vincristine sulfate
2 A Groupwide Randomized Phase II Window Study of Two Different Schedules of Irinotecan in Combination With Vincristine And Pilot Assessment of Safety and Efficacy of Tirapazamine Combined With Multiagent Chemotherapy for First Relapse or Progressive Disease in Rhabdomyosarcoma and Related Tumors Completed NCT00025363 Phase 2 vincristine sulfate;irinotecan hydrochloride;cyclophosphamide;doxorubicin hydrochloride;ifosfamide;etoposide;tirapazamine

Search NIH Clinical Center for Ectomesenchymoma

Genetic Tests for Ectomesenchymoma

Anatomical Context for Ectomesenchymoma

MalaCards organs/tissues related to Ectomesenchymoma:

40
Bone, Prostate, Kidney, Lymph Node, Brain, Lung, Medulla Oblongata

Publications for Ectomesenchymoma

Articles related to Ectomesenchymoma:

(show top 50) (show all 70)
# Title Authors PMID Year
1
Epidemiology, Incidence, and Survival of Rhabdomyosarcoma Subtypes: SEER and ICES Database Analysis. 61
31161653 2019
2
Malignant Ectomesenchymoma: A Potential Pitfall of Diagnosis in the Spectrum of Pediatric Small Blue Round Cell Tumors. 61
28968263 2019
3
Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature. 61
30907031 2019
4
Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor. 61
28994342 2018
5
Robot-assisted retroperitoneal lymph node dissection (RA-RPLND) in the adolescent population. 61
28262537 2017
6
Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma. 61
26872011 2016
7
An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis. 61
26793539 2015
8
A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum. 61
26339431 2015
9
Ectomesenchymoma with embryonal rhabdomyosarcoma and ganglioneuroma, arising in association with benign triton tumor of the tongue. 61
24650353 2014
10
Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. 61
25405050 2014
11
Malignant pediatric brain tumor of primitive small round cell proliferation with bland-looking mesenchymal spindle cell elements. 61
22684841 2013
12
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS). 61
22535600 2013
13
Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. 61
23174743 2012
14
Ectomesenchymoma with t(1;12)(p32;p13) evolving from embryonal rhabdomyosarcoma shows no rearrangement of ETV6. 61
21803398 2012
15
Malignant ectomesenchymoma of the nasal cavity. 61
22563163 2011
16
A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri. 61
21194292 2011
17
Malignant ectomesenchymoma of the paranasal sinuses with proptosis. 61
22026142 2011
18
Retroperitoneal ectomesenchymoma: a rare childhood tumor. 61
20090275 2010
19
[Malignant ectomesenchymoma of left vulva in childhood: report of a case]. 61
20078983 2009
20
Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. 61
18043289 2007
21
Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. 61
17895746 2007
22
Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor. 61
17431644 2007
23
Malignant ectomesenchymoma of the nasal cavity. 61
17395109 2007
24
A neonate with malignant ectomesenchymoma. 61
16363352 2005
25
A 10-month-old boy with a large pineal tumor. 61
16196394 2005
26
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. 61
15747131 2005
27
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. 61
15735865 2005
28
Ectomesenchymoma: case report and review of the literature. 61
15074381 2004
29
Extensive posttreatment ganglioneuromatous differentiation of rhabdomyosarcoma: malignant ectomesenchymoma in an infant. 61
12469232 2003
30
Malignant ectomesenchymoma of the nose. Case report and review of the literature. 61
12624508 2003
31
Rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report. 61
12461409 2002
32
Primary malignant ectomesenchymoma of the kidney. 61
12383226 2002
33
Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. 61
12161212 2002
34
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. 61
11787870 2002
35
[Pelvic malignant ectomesenchymoma: a case report]. 61
11685134 2001
36
Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. 61
11297080 2001
37
Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. 61
10503746 1999
38
Malignant ectomesenchymoma. Case report and review of the literature. 61
10494059 1999
39
Ectomesenchymoma of the prostate: histological diagnostic criteria. 61
9914362 1999
40
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. 61
9544170 1998
41
Ectomesenchymoma of the medulla oblongata in a calf. 61
9421949 1997
42
Immunohistochemical and molecular genetic approaches to soft tissue tumor diagnosis: a primer. 61
9344317 1997
43
Ectomesenchymoma: one or two tumors? Case report and review of the literature. 61
9314261 1997
44
Radiologic-Pathologic Conference of Children's Hospital Boston: intraconal mass in the orbit of an infant. 61
9252435 1997
45
Primary malignant ectomesenchymoma of the orbit. 61
9065587 1997
46
Malignant ectomesenchymoma in childhood. 61
9025858 1996
47
Ectomesenchymal hamartoma (benign "ectomesenchymoma") of the VIIIth nerve: case report. 61
8584163 1995
48
Relationship of DNA ploidy to histology and prognosis in rhabdomyosarcoma. Comparison of flow cytometry and image analysis. 61
7526971 1994
49
[Biopathology of Ewing's sarcoma]. 61
7605216 1994
50
Plasma neuropeptide Y in healthy children: influence of age, anaesthesia and the establishment of an age-adjusted reference interval. 61
8025403 1994

Variations for Ectomesenchymoma

ClinVar genetic disease variations for Ectomesenchymoma:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 MSH2 NM_000251.2(MSH2):c.2732T>G (p.Leu911Arg)SNV Conflicting interpretations of pathogenicity 91039 rs41295182 2:47710015-47710015 2:47482876-47482876

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 10.1 HRAS FOXO1

GO Terms for Ectomesenchymoma

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

# Name GO ID Score Top Affiliating Genes
1 protein-DNA complex GO:0032993 8.96 MYOG HMGA2
2 senescence-associated heterochromatin focus GO:0035985 8.62 HMGA2 HMGA1

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 9.67 MYOG HMGA2 HMGA1 FOXO1
2 positive regulation of transcription by RNA polymerase II GO:0045944 9.65 MYOG HRAS HMGA2 HMGA1 FOXO1
3 base-excision repair GO:0006284 9.43 HMGA2 HMGA1
4 regulation of heart contraction GO:0008016 9.4 S100A1 DES
5 positive regulation of cell cycle arrest GO:0071158 9.32 MYOG HMGA2
6 positive regulation of cellular senescence GO:2000774 9.16 HMGA2 HMGA1
7 senescence-associated heterochromatin focus assembly GO:0035986 8.96 HMGA2 HMGA1
8 oncogene-induced cell senescence GO:0090402 8.62 HMGA2 HMGA1

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.85 S100A1 MYOG MSH2 HRAS HMGA2 HMGA1
2 DNA-(apurinic or apyrimidinic site) endonuclease activity GO:0003906 8.96 HMGA2 HMGA1
3 AT DNA binding GO:0003680 8.62 HMGA2 HMGA1

Sources for Ectomesenchymoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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