MTT
MCID: MLG041
MIFTS: 38

Malignant Triton Tumor (MTT)

Categories: Cancer diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Malignant Triton Tumor

MalaCards integrated aliases for Malignant Triton Tumor:

Name: Malignant Triton Tumor 11 58 14 75
Malignant Peripheral Nerve Sheath Tumor with Rhabdomyosarcomatous Differenciation 58
Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation 71
Malignant Peripheral Nerve Sheath Neoplasm with Rhabdomyosarcoma 11
Mpnst with Rhabdomyosarcomatous Differentiation 58
Mpnst with Rhabdomyoblastic Differentiation 11
Mpnst with Rhabdomyosarcoma 11
Malignant Triton Tumour 11
Mtt 58

Characteristics:


Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:6707
NCIt 49 C4335
SNOMED-CT 68 354002
UMLS via Orphanet 72 C0334616
Orphanet 58 ORPHA252212
UMLS 71 C0334616

Summaries for Malignant Triton Tumor

Orphanet: 58 Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST; see this term) characterized histopathologically by focal rhabdomyoblastic differentiation.

MalaCards based summary: Malignant Triton Tumor, also known as malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation, is related to malignant peripheral nerve sheath tumor and neurofibromatosis, type i. An important gene associated with Malignant Triton Tumor is HTRA1 (HtrA Serine Peptidase 1), and among its related pathways/superpathways are Mesenchymal Stem Cells and Lineage-specific Markers and Myogenesis. The drugs Ifosfamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include small intestine, lung and spinal cord, and related phenotypes are growth/size/body region and embryo

Wikipedia: 75 Malignant triton tumor (MTT) is a relatively rare, aggressive tumor made up of both malignant schwannoma... more...

Related Diseases for Malignant Triton Tumor

Diseases related to Malignant Triton Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 453)
# Related Disease Score Top Affiliating Genes
1 malignant peripheral nerve sheath tumor 30.8 SOX10 NF1 MYOG CD34 B3GAT1
2 neurofibromatosis, type i 30.7 NF1 ENO2 CD34
3 atypical neurofibroma 30.3 SOX10 NF1
4 neurofibroma 30.3 SOX10 NF1 MLANA CD34
5 botryoid rhabdomyosarcoma 30.2 MYOG MYOD1 MB
6 trigeminal nerve neoplasm 30.2 NF1 CD34
7 malignant fibrous histiocytoma 30.0 MYOG MYOD1 MB
8 neurilemmoma 30.0 SOX10 NF1 MLANA ENO2 CD34
9 rhabdomyosarcoma 30.0 NF1 MYOG MYOD1 MB ENO2
10 liposarcoma 29.8 NF1 MYOG MB CD34
11 malignant mesenchymoma 29.7 MYOG MYOD1 MB CD34
12 fibrous histiocytoma 29.7 SOX10 MLANA CD34
13 sarcoma 29.7 SS18 MYOG MYOD1 MB ENO2
14 tuberous sclerosis 29.6 NF1 MLANA ENO2
15 large congenital melanocytic nevus 29.6 SOX10 MLANA
16 sarcoma, synovial 29.4 TLE1 SS18 NF1 MYOG CD34
17 ewing sarcoma 29.2 SS18 NF1 MYOG MYOD1 ENO2
18 paraganglioma 29.1 SOX10 NF1 MLANA ENO2
19 cutaneous fibrous histiocytoma 29.0 SOX10 MLANA ENO2 CD34
20 embryonal rhabdomyosarcoma 28.7 SS18 NF1 MYOG MYOD1 MB ENO2
21 wilms tumor 1 28.5 NF1 MYOG MYOD1 MLANA ENO2 CD34
22 spindle cell sarcoma 28.1 TLE1 SS18 SOX10 NF1 MYOG MYOD1
23 neurofibromatosis 10.8
24 neurofibromatosis-noonan syndrome 10.8
25 pituitary tumors 10.5
26 rare tumor 10.4
27 malignant peripheral nerve sheath tumor with perineurial differentiation 10.3 SH3PXD2A HTRA1
28 cafe-au-lait spots, multiple 10.3
29 oral rhabdomyosarcoma 10.3 MYOG MYOD1
30 gallbladder sarcoma 10.3 MYOG MYOD1
31 prostate rhabdomyosarcoma 10.3 MYOG MYOD1
32 medullomyoblastoma 10.3 MYOG MB
33 ornithosis 10.3 MB HTRA1
34 amyloidosis 10.2
35 acoustic neuroma 10.2
36 vagina leiomyosarcoma 10.2 MYOG MLANA
37 conjunctival nevus 10.2 NF1 MLANA
38 leukemia, acute myeloid 10.2
39 gastric cancer 10.2
40 leukemia 10.2
41 choroid cancer 10.2 NF1 MLANA
42 malignant dermis tumor 10.2 NF1 MLANA
43 vulvar melanoma 10.2 NF1 MLANA
44 malignant skin fibrous histiocytoma 10.2 NF1 MLANA
45 progressive relapsing multiple sclerosis 10.2 MYOG MYOD1
46 schwannoma of twelfth cranial nerve 10.2 NF1 MLANA
47 hypoglossal nerve disease 10.2 NF1 MLANA
48 inflammatory leiomyosarcoma 10.2 NF1 MYOG MYOD1
49 myeloid leukemia 10.2
50 acral lentiginous melanoma 10.2 NF1 MLANA

Graphical network of the top 20 diseases related to Malignant Triton Tumor:



Diseases related to Malignant Triton Tumor

Symptoms & Phenotypes for Malignant Triton Tumor

MGI Mouse Phenotypes related to Malignant Triton Tumor:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.85 ENO2 HTRA1 MAML3 MB MYOD1 MYOG
2 embryo MP:0005380 9.5 MAML3 MB MYOG NF1 SH3PXD2A SOX10
3 respiratory system MP:0005388 9.17 ENO2 MB MYOD1 MYOG NF1 SH3PXD2A

Drugs & Therapeutics for Malignant Triton Tumor

Drugs for Malignant Triton Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
4
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
5
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
6
Palifosfamide Investigational Phase 2, Phase 3 31645-39-3 100427
7 Liposomal doxorubicin Phase 2, Phase 3
8 Anti-Bacterial Agents Phase 2, Phase 3
9 Alkylating Agents Phase 2, Phase 3
10 Antineoplastic Agents, Alkylating Phase 2, Phase 3
11 Antibiotics, Antitubercular Phase 2, Phase 3
12
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NSC# 737754) Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
2 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Unknown status NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI

Search NIH Clinical Center for Malignant Triton Tumor

Genetic Tests for Malignant Triton Tumor

Anatomical Context for Malignant Triton Tumor

Organs/tissues related to Malignant Triton Tumor:

MalaCards : Small Intestine, Lung, Spinal Cord, Thyroid, Kidney, Pituitary, Liver

Publications for Malignant Triton Tumor

Articles related to Malignant Triton Tumor:

(show top 50) (show all 173)
# Title Authors PMID Year
1
Malignant Triton tumor of the retroperitoneum in a male unaffected by neurofibromatosis 1: A case report and literature review. 62
35725797 2022
2
How to Treat Metastatic Malignant Triton Tumor in an Adolescent. 62
36445979 2022
3
Computed tomography imaging features of malignant 'triton' tumor to facilitate its clinical diagnosis: report of two cases. 62
35836141 2022
4
Liver metastasis 13 years after the resection of a malignant triton tumor originating in the duodenum. 62
35669906 2022
5
Case report: Primary intracranial EWs/PNET in adults: Clinical experience and literature review. 62
36313718 2022
6
Giant malignant Triton tumour of the posterior mediastinum. 62
34041530 2021
7
Malignant triton tumor of the kidney in a child: A case report. 62
34358962 2021
8
Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location. 62
34007400 2021
9
Loss of H3K27me3 occurs in a large subset of embryonal rhabdomyosarcomas: Immunohistochemical and molecular analysis of 25 cases. 62
33770660 2021
10
[Malignant Triton tumour: A case report]. 62
33931246 2021
11
A Rare Case of Progressive Malignant Triton Tumor With Rare Somatic Mutation in TSC2 Gene. 62
34083294 2021
12
Malignant triton tumour (MTT) of the lung with metastasis to the proximal femur. 62
33542007 2021
13
The importance of surgical margins in malignant Triton tumour of the trunk and extremities. 62
33376551 2021
14
Malignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature. 62
33791136 2021
15
Triton tumor of the orbit. 62
31746248 2020
16
[Focus on oncologic safety and function: Primary defect reconstruction of a malignant Triton tumor of the sciatic nerve sheath with myocutaneous free flap surgery]. 62
32120413 2020
17
Retroperitoneal malignant triton tumor in an infant: a case report and literature review. 62
32953555 2020
18
Elbow flexion reconstruction after arm-sparing excision for high-grade triton sarcoma: a case report. 62
32620140 2020
19
Malignant triton tumor below the peritoneal reflection: a case report. 62
32595924 2020
20
Worsening Hemoptysis in a Rare Intrapulmonary Triton Tumor Mimicking Lung Cancer. 62
31400331 2020
21
Malignant triton tumor of the rectum - A case report and review of the literature. 62
33207422 2020
22
Malignant Triton Tumor Diagnosed on Fine Needle Aspiration Cytology. 62
31741582 2019
23
A series of 10 malignant triton tumors in one institution. 62
31490366 2019
24
Malignant triton tumor of the left thoracic cavity: a case report. 62
31528328 2019
25
Malignant triton tumor diagnosed twelve years after radiosurgically treated vestibular schwannoma. 62
31176932 2019
26
Malignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor. 62
28924675 2019
27
Expert's comment concerning Grand Rounds case entitled "Malignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor" by S. Ghailane et al. (Eur Spine J; 2017: DOI 10.1007/s00586-017-5277-2). 62
29671111 2019
28
Teaching NeuroImages: Intracranial malignant triton tumor: An uncommon location of a rare tumor. 62
30886081 2019
29
Mediastinal malignant triton tumor: A rare case series and review of literature. 62
31494456 2019
30
Malignant Triton Tumor in a Child: Case Report and Literature Review. 62
31920385 2019
31
Malignant Triton tumour of the maxilla: A case report. 62
29908757 2018
32
Malignant triton tumor of trigeminal nerve-case report. 62
29279963 2018
33
Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report. 62
29110923 2018
34
Malignant triton tumor of the duodenum: report of a case. 62
31149515 2018
35
A rare case of intracranial malignant triton tumor arising in the middle cranial fossa: a case report and review of the literature. 62
30509044 2018
36
[A Rare Case of Abdominal Malignant Triton Tumor]. 62
29394776 2017
37
Malignant Triton Tumor (Malignant Peripheral Nerve Sheath Tumor With Rhabdomyoblastic Differentiation) Occurring in a Vascularized Free Flap Reconstruction Graft. 62
28387148 2017
38
A Case of Retroperitoneal Malignant Triton Tumor in a Nigerian Boy. 62
29089742 2017
39
Malignant Triton tumor: a rare cause of sciatic pain and foot drop. 62
28884110 2017
40
Malignant triton tumor of the lung, infiltrating the left atrium and left ventricle, with metastasis to the small intestine. 62
28515751 2017
41
Malignant Triton Tumor of the Kidney. New Location Not Previously Reported. 62
26159374 2017
42
Radical resection and improvised surgical reconstruction for a rare malignant triton tumour of intercostal nerve in a patient with neurofibromatosis type 1. 62
27932435 2016
43
A case of recurrent malignant triton tumor successfully treated with radiotherapy. 62
27146005 2016
44
Insidious malignant triton tumor of the chest wall with late flare-up. 62
28757738 2016
45
Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation. 62
26371783 2016
46
A rare case of recurrent malignant triton tumor in a male with NF1: Case report and mini-review. 62
26975033 2016
47
Analysis of clinical features and prognosis of malignant triton tumor: A report of two cases and literature review. 62
26788168 2015
48
Primary epididymis malignant triton tumor: case report and review of the literature. 62
26392076 2015
49
Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous and Glandular Elements: Rare Epithelial Differentiation in a Triton Tumor. 62
25911565 2015
50
Unusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. 62
25879014 2015

Variations for Malignant Triton Tumor

Expression for Malignant Triton Tumor

Search GEO for disease gene expression data for Malignant Triton Tumor.

Pathways for Malignant Triton Tumor

Pathways related to Malignant Triton Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.78 MYOG MYOD1 CD34
2 10.43 MYOG MYOD1

GO Terms for Malignant Triton Tumor

Biological processes related to Malignant Triton Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of skeletal muscle fiber development GO:0048743 9.56 MYOG MYOD1
2 negative regulation of Schwann cell proliferation GO:0010626 9.46 SOX10 NF1
3 skeletal muscle tissue development GO:0007519 9.43 NF1 MYOG MYOD1
4 myotube differentiation GO:0014902 9.32 MYOG MYOD1
5 muscle cell fate commitment GO:0042693 8.92 MYOG MYOD1

Molecular functions related to Malignant Triton Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription activator activity GO:0001216 8.92 SOX10 MYOG MYOD1

Sources for Malignant Triton Tumor

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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