CHDM
MCID: CHR072
MIFTS: 64

Chordoma (CHDM)

Categories: Bone diseases, Cancer diseases, Endocrine diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Chordoma

MalaCards integrated aliases for Chordoma:

Name: Chordoma 57 12 76 53 25 59 75 29 13 55 44 15 73
Chdm 57 25 75
Notochordal Sarcoma 25 59
Notochordoma 12 25
Chordoma, Susceptibility to 57
Chordoepithelioma 25
Chordocarcinoma 25

Characteristics:

Orphanet epidemiological data:

59
chordoma
Inheritance: Autosomal dominant,Not applicable; Prevalence: <1/1000000 (United States); Age of onset: Adult; Age of death: any age;

OMIM:

57
Inheritance:
autosomal dominant

Miscellaneous:
median age at diagnosis, 59 years


HPO:

32
chordoma:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM 57 215400
Disease Ontology 12 DOID:3302
MeSH 44 D002817
NCIt 50 C2947
SNOMED-CT 68 50007008
Orphanet 59 ORPHA178
MESH via Orphanet 45 D002817
UMLS via Orphanet 74 C0008487
ICD10 via Orphanet 34 C76.7
MedGen 42 C0008487
SNOMED-CT via HPO 69 263681008
UMLS 73 C0008487

Summaries for Chordoma

NIH Rare Diseases : 53 A chordoma is a rare tumor that develops from cells of the notochord, a structure that is present in the developing embryo and is important for the development of the spine. The notochord usually disappears before birth, though a few cells may remain embedded in the bones of the spine or at the base of the skull. Chordomas typically present in adults between the ages of 40 and 70 and can occur anywhere along the spine. About half of all chordomas occur at the bottom of the spine (sacrum); about one third occur at the base of the skull. The remaining cases of chordomas form in the spine at the level of the neck, chest, or other parts of the lower back. Chordomas grow slowly, extending gradually into the surrounding bone and soft tissue. The actual symptoms depend on the location of the chordoma. A chordoma that occurs at the base of the spine may cause problems with bladder and bowel function. A chordoma at the base of the skull may lead to double vision and headaches.  In many cases, the cause of the chordoma remains unknown. Recent studies have shown that changes in the T gene have been associated with chordomas in a small set of families. In these families an inheritedduplication of the T gene is associated with an increased risk of developing a chordoma. Duplications of the T gene have also been identified in people with chordoma who have no history of the tumor in their family, but in these cases the changes occur only in the tumor cells and are not inherited. The current treatment is often the surgical removal of the tumor, followed by radiotherapy.

MalaCards based summary : Chordoma, also known as chdm, is related to spinal chordoma and chondroid chordoma. An important gene associated with Chordoma is CHDM (Chordoma), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Dasatinib and Sunitinib have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and brain, and related phenotypes are abnormality of the vertebral column and abnormality of the head

Disease Ontology : 12 A notochordal cancer that derives from cellular remnants of the notochord.

Genetics Home Reference : 25 A chordoma is a rare type of cancerous tumor that can occur anywhere along the spine, from the base of the skull to the tailbone. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. They often recur after treatment, and in about 40 percent of cases the cancer spreads (metastasizes) to other areas of the body, such as the lungs.

OMIM : 57 Chordomas are rare, clinically malignant tumors derived from notochordal remnants. They occur along the length of the spinal axis, predominantly in the sphenooccipital, vertebral, and sacrococcygeal regions. They are characterized by slow growth, local destruction of bone, extension into adjacent soft tissues, and, rarely, distant metastatic spread (Stepanek et al., 1998). The incidence of chordoma is age-dependent, with fewer than 5% occurring in children and adolescents (summary by McMaster et al., 2011). (215400)

UniProtKB/Swiss-Prot : 75 Chordoma: Rare, clinically malignant tumors derived from notochordal remnants. They occur along the length of the spinal axis, predominantly in the sphenooccipital, vertebral and sacrococcygeal regions. They are characterized by slow growth, local destruction of bone, extension into adjacent soft tissues and rarely, distant metastatic spread.

Wikipedia : 76 Chordoma is a rare slow-growing neoplasm thought to arise from cellular remnants of the notochord. The... more...

Related Diseases for Chordoma

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

(show top 50) (show all 308)
# Related Disease Score Top Affiliating Genes
1 spinal chordoma 33.8 EGFR MET PDGFRA S100B
2 chondroid chordoma 33.6 CDH1 CEACAM3 MUC1 S100A1 SMARCB1 VIM
3 parachordoma 30.4 DES GFAP KRT7 MUC1 VIM
4 fibrous histiocytoma 30.3 DES S100B VIM
5 myxopapillary ependymoma 30.3 GFAP MUC1 S100B VIM
6 malignant fibroxanthoma 30.3 DES MUC1 S100B VIM
7 non-functioning pituitary adenoma 30.2 MUC1 S100B
8 chordoid meningioma 30.2 GFAP MUC1 S100B VIM
9 ependymoma 30.2 EGFR GFAP MUC1 S100B VIM
10 pleomorphic adenoma 30.1 GFAP MUC1 S100B VIM
11 giant cell tumor 30.1 CTSK DES MUC1 VIM
12 alveolar soft part sarcoma 30.1 DES MET VIM
13 hemangioblastoma 30.1 GFAP MUC1 S100B
14 adenocarcinoma 30.0 CDH1 CDKN2A EGFR MUC1 PTEN
15 rhabdoid meningioma 30.0 GFAP SMARCB1 VIM
16 neurofibroma 29.9 CDKN2A MUC1 PDGFRA S100B
17 myoepithelioma 29.9 GFAP S100A1 S100B SMARCB1 VIM
18 hemangiopericytoma, malignant 29.9 DES MUC1 S100B VIM
19 colorectal adenocarcinoma 29.9 CDH1 EGFR KRT7
20 uterine carcinosarcoma 29.9 CDH1 EGFR PTEN
21 chondromyxoid fibroma 29.8 CDKN2A DES S100A1 VIM
22 epithelioid sarcoma 29.8 KRT7 MUC1 SMARCB1 VIM
23 carcinosarcoma 29.7 DES KRT7 MUC1 PTEN VIM
24 renal cell carcinoma, nonpapillary 29.5 CDH1 EGFR KRT19 KRT7 MET MUC1
25 clear cell renal cell carcinoma 29.5 CDH1 KRT7 MET MUC1 PTEN VIM
26 clivus chordoma 12.5
27 clivus chondroid chordoma 12.5
28 sacrum chordoma 12.4
29 chondrosarcoma 10.6
30 degos 'en cocarde' erythrokeratoderma 10.5
31 localized hypertrophic neuropathy 10.4 MUC1 S100B
32 sclerosing perineurioma 10.4 MUC1 S100B
33 bednar tumor 10.4 S100B VIM
34 sinonasal undifferentiated carcinoma 10.4 CEACAM3 MUC1
35 primary hepatic neuroendocrine carcinoma 10.4 S100B VIM
36 spindle cell thymoma 10.4 DES MUC1
37 spindle cell lipoma 10.4 DES VIM
38 angiolipoma 10.3 DES VIM
39 epulis 10.3 DES VIM
40 infantile digital fibromatosis 10.3 DES VIM
41 lymphangiomatosis 10.3 DES VIM
42 breast scirrhous carcinoma 10.3 EGFR MUC1
43 malignant triton tumor 10.3 DES S100B
44 solid adenocarcinoma with mucin production 10.3 KRT7 MUC1
45 endosalpingiosis 10.3 KRT7 MUC1
46 gliomatosis peritonei 10.3 DES GFAP
47 bile duct mucoepidermoid carcinoma 10.3 KRT7 MUC1
48 middle ear adenoma 10.3 KRT7 MUC1
49 malignant iris melanoma 10.3 S100A1 S100B
50 malignant ectomesenchymoma 10.3 DES S100A1

Graphical network of the top 20 diseases related to Chordoma:



Diseases related to Chordoma

Symptoms & Phenotypes for Chordoma

Symptoms via clinical synopsis from OMIM:

57
Skeletal Skull:
sphenooccipital chordoma
nasopharyngeal chordoma

Skeletal Spine:
vertebral chordoma
sacrococcygeal chordoma


Clinical features from OMIM:

215400

Human phenotypes related to Chordoma:

32
# Description HPO Frequency HPO Source Accession
1 abnormality of the vertebral column 32 HP:0000925
2 abnormality of the head 32 HP:0000234
3 chordoma 32 HP:0010762

GenomeRNAi Phenotypes related to Chordoma according to GeneCards Suite gene sharing:

26 (show all 11)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00055-A-2 10.18 EGFR
2 Decreased viability GR00173-A 10.18 PDGFRA
3 Decreased viability GR00221-A-1 10.18 CDH1 CDKN2A EGFR PDGFRA SMARCB1
4 Decreased viability GR00221-A-2 10.18 SMARCB1
5 Decreased viability GR00221-A-3 10.18 CDKN2A PDGFRA SMARCB1
6 Decreased viability GR00221-A-4 10.18 CDKN2A EGFR PDGFRA
7 Decreased viability GR00301-A 10.18 CDH1
8 Decreased viability GR00381-A-1 10.18 SMARCB1
9 Decreased viability GR00402-S-2 10.18 CDH1 CDKN2A EGFR PDGFRA SMARCB1
10 Decreased viability in esophageal squamous lineage GR00235-A 9.65 CDH1 CDKN2A CEACAM3 DES GFAP KRT7
11 Reduced mammosphere formation GR00396-S 9.28 CDH1 CTSK DES EGFR KRT7 PTEN

MGI Mouse Phenotypes related to Chordoma:

46 (show all 18)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.43 CDH1 CDKN2A CTSK DES EGFR GFAP
2 cardiovascular system MP:0005385 10.41 CDH1 CDKN2A DES EGFR GFAP KRT19
3 homeostasis/metabolism MP:0005376 10.41 CDH1 CDKN2A CTSK DES EGFR GFAP
4 behavior/neurological MP:0005386 10.35 CDKN2A DES GFAP LGALS3 MET PDGFRA
5 digestive/alimentary MP:0005381 10.33 CDH1 CDKN2A CTSK EGFR GFAP KRT19
6 growth/size/body region MP:0005378 10.3 CDH1 CDKN2A CTSK EGFR GFAP KRT19
7 mortality/aging MP:0010768 10.27 CDH1 CDKN2A DES EGFR GFAP KRT19
8 embryo MP:0005380 10.24 CDH1 CDKN2A EGFR KRT19 MET PDGFRA
9 immune system MP:0005387 10.24 CDH1 CDKN2A CTSK EGFR GFAP LGALS3
10 nervous system MP:0003631 10.15 CDKN2A CTSK EGFR GFAP KRT19 LGALS3
11 integument MP:0010771 10.13 CDH1 CDKN2A EGFR KRT19 LGALS3 PDGFRA
12 muscle MP:0005369 10.13 CDKN2A DES EGFR GFAP MET PDGFRA
13 neoplasm MP:0002006 10.06 CDH1 CDKN2A EGFR KRT19 LGALS3 MET
14 normal MP:0002873 9.96 CDH1 EGFR GFAP KRT19 MET PDGFRA
15 no phenotypic analysis MP:0003012 9.87 CDH1 CDKN2A EGFR KRT19 MET PDGFRA
16 renal/urinary system MP:0005367 9.7 EGFR KRT7 LGALS3 MET PDGFRA PTEN
17 reproductive system MP:0005389 9.65 CDH1 CDKN2A EGFR KRT19 LGALS3 PDGFRA
18 respiratory system MP:0005388 9.28 CDKN2A CTSK EGFR LGALS3 MET PDGFRA

Drugs & Therapeutics for Chordoma

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

(show top 50) (show all 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
2
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
3
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 6055-19-2, 50-18-0 2907
4
Palbociclib Approved, Investigational Phase 2 571190-30-2 11431660 5330286 5005498
5
Nivolumab Approved Phase 2,Phase 1 946414-94-4
6
Trioxsalen Approved Phase 2,Phase 1 3902-71-4 5585
7
Cobalt Approved, Experimental Phase 2 7440-48-4 104729
8
Cetuximab Approved Phase 1, Phase 2 205923-56-4 56842117 2333
9
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
10
Doxil Approved June 1999 Phase 1, Phase 2 31703
11 Imatinib Mesylate Phase 2,Phase 1 220127-57-1 123596
12 Protein Kinase Inhibitors Phase 2,Phase 1
13 Immunologic Factors Phase 2,Phase 1,Not Applicable
14 Immunosuppressive Agents Phase 2,Phase 1
15 Angiogenesis Modulating Agents Phase 2
16 Angiogenesis Inhibitors Phase 2
17 Antineoplastic Agents, Alkylating Phase 1, Phase 2
18 Alkylating Agents Phase 1, Phase 2
19 Antirheumatic Agents Phase 1, Phase 2
20 lysine Phase 2,Phase 1
21 Antibodies Phase 2,Phase 1
22 Antibodies, Monoclonal Phase 2,Phase 1
23 Immunoglobulins Phase 2,Phase 1
24 Photosensitizing Agents Phase 2,Phase 1
25 Hematoporphyrin Derivative Phase 2,Phase 1
26 Dermatologic Agents Phase 2,Phase 1
27 Hematoporphyrins Phase 2,Phase 1
28 Dihematoporphyrin Ether Phase 2,Phase 1
29 Ether Phase 2,Phase 1
30 Vaccines Phase 2,Phase 1
31 Brewer's Yeast Phase 2
32 Pharmaceutical Solutions Phase 2
33 Topoisomerase Inhibitors Phase 1, Phase 2
34 Anti-Bacterial Agents Phase 1, Phase 2
35 Antibiotics, Antitubercular Phase 1, Phase 2
36 Immunoglobulin G Phase 2
37
Panobinostat Approved, Investigational Phase 1 404950-80-7 6918837
38
Busulfan Approved, Investigational Phase 1 55-98-1 2478
39
Melphalan Approved Phase 1 148-82-3 4053 460612
40
Amifostine Approved, Investigational Phase 1 20537-88-6 2141
41
Lenograstim Approved, Investigational Phase 1 135968-09-1
42
Thiotepa Approved, Investigational Phase 1 52-24-4 5453
43 Mitogens Phase 1
44 Histone Deacetylase Inhibitors Phase 1
45 Adjuvants, Immunologic Phase 1,Not Applicable
46 Protective Agents Phase 1,Not Applicable
47 Liver Extracts Phase 1
48 Radiation-Protective Agents Phase 1
49
Povidone Approved Not Applicable 9003-39-8
50
Hyaluronic acid Approved, Vet_approved Not Applicable 9004-61-9 53477741

Interventional clinical trials:

(show all 47)
# Name Status NCT ID Phase Drugs
1 Stereotactic Body Radiotherapy for Head and Neck Tumors Completed NCT01344356 Phase 4
2 Trial of Proton Versus Carbon Ion Radiation Therapy in Patients With Chordoma of the Skull Base Recruiting NCT01182779 Phase 3
3 Proton Radiation for Chordomas and Chondrosarcomas Unknown status NCT01449149 Phase 1, Phase 2
4 Efficacy and Safety of Imatinib in Chordoma Completed NCT00150072 Phase 2 imatinib
5 Charged Particle RT for Chordomas and Chondrosarcomas of the Base of Skull or Cervical Spine Completed NCT00592748 Phase 1, Phase 2
6 Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
7 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
8 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
9 Improvement of Local Control in Skull Base, Spine and Sacral Chordomas Treated by Surgery and Protontherapy Targeting Hypoxic Cells Revealed by [18F]FAZA) PET/CT Tracers Recruiting NCT02802969 Phase 2 18F FAZA
10 Ion Irradiation of Sacrococcygeal Chordoma Recruiting NCT01811394 Phase 2
11 CDK4/6 Inhibition in Locally Advanced/Metastatic Chordoma Recruiting NCT03110744 Phase 2 Palbociclib
12 Afatinib in Locally Advanced and Metastatic Chordoma Recruiting NCT03083678 Phase 2 Afatinib
13 BN Brachyury and Radiation in Chordoma Recruiting NCT03595228 Phase 2
14 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2 Tazemetostat
15 Immune Checkpoint Inhibitor Nivolumab in People With Select Rare CNS Cancers Recruiting NCT03173950 Phase 2 Nivolumab
16 Nivolumab and Ipilimumab in Treating Patients With Rare Tumors Recruiting NCT02834013 Phase 2
17 A Phase II Study Evaluating Efficacy and Safety of Regorafenib in Patients With Metastatic Bone Sarcomas Recruiting NCT02389244 Phase 2 Regorafenib;Placebo
18 QUILT-3.011 Phase 2 Yeast-Brachyury Vaccine Chordoma Active, not recruiting NCT02383498 Phase 2
19 Proton Beam Therapy for Chordoma Patients Active, not recruiting NCT00496119 Phase 2
20 Photodynamic Therapy (PDT) For Recurrent High Grade Gliomas Active, not recruiting NCT01966809 Phase 2 Photofrin photodynamic therapy.
21 QUILT-3.091 NANT Chordoma Vaccine vs Radiation in Subjects With Unresectable Chordoma. Not yet recruiting NCT03647423 Phase 1, Phase 2 Avelumab;Cetuximab;Cyclophosphamide
22 Nivolumab and Relatlimab in Treating Participants With Advanced Chordoma Not yet recruiting NCT03623854 Phase 2
23 Study of Imatinib, a Platelet-derived Growth Factor Receptor Inhibitor, and LBH589, a Histone Deacetylase Inhibitor, in the Treatment of Newly Diagnosed and Recurrent Chordoma Unknown status NCT01175109 Phase 1 Imatinib + LBH589
24 HSV1716 in Patients With Non-Central Nervous System (Non-CNS) Solid Tumors Completed NCT00931931 Phase 1
25 Safety Study of Intratumoral Injection of Clostridium Novyi-NT Spores to Treat Patients With Solid Tumors That Have Not Responded to Standard Therapies Completed NCT01924689 Phase 1
26 Imatinib Mesylate And Cyclophosphamide In Metronomic Administration: Dose Escalation Study Of Imatinib Mesylate Completed NCT01046487 Phase 1 Imatinib mesylate, Cyclophosphamide (Dosing level 1 );Imatinib mesylate, Cyclophosphamide (Dosing level 2);Imatinib mesylate, Cyclophosphamide (Dosing level 3)
27 Nivolumab With or Without Stereotactic Radiosurgery in Treating Patients With Recurrent, Advanced, or Metastatic Chordoma Recruiting NCT02989636 Phase 1
28 A Study of FAZ053 Single Agent and in Combination With PDR001 in Patients With Advanced Malignancies. Recruiting NCT02936102 Phase 1 FAZ053;PDR001
29 Photodynamic Therapy (PDT) for Recurrent Pediatric Brain Tumors Recruiting NCT01682746 Phase 1 Photofrin (porfimer sodium) & photodynamic therapy.
30 Nilotinib With Radiation for High Risk Chordoma Active, not recruiting NCT01407198 Phase 1 Nilotinib
31 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Active, not recruiting NCT02601937 Phase 1 Tazemetostat
32 Amifostine to Protect From Side Effects of PSCT in Treating Patients With Solid Tumors Terminated NCT00003926 Phase 1 amifostine trihydrate;busulfan;filgrastim;melphalan;thiotepa
33 Polyvinylpyrrolidone-Sodium Hyaluronate Gel in Reducing Pain From Oral Mucositis in Young Patients With Cancer Unknown status NCT00349024 Not Applicable polyvinylpyrrolidone-sodium hyaluronate gel
34 Genetic Aspects of Chordoma: A Collaboration With SEER Registries to Identify Chordoma Families Completed NCT00341627
35 Chordoma Family Study Completed NCT00410670
36 Hypoxia-positron Emission Tomography (PET) and Intensity Modulated Proton Therapy (IMPT) Dose Painting in Patients With Chordomas Completed NCT00713037 Not Applicable
37 Proton Therapy for Chordomas and/or Chondrosarcomas Completed NCT00797602
38 Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas Completed NCT01567046
39 Genetic Clues to Chordoma Etiology: A Protocol to Identify Sporadic Chordoma Patients for Studies of Cancer-Susceptibility Genes Recruiting NCT01200680
40 Sacral Chordoma: Surgery Versus Definitive Radiation Therapy in Primary Localized Disease Recruiting NCT02986516 Not Applicable
41 Randomized Carbon Ions vs Standard Radiotherapy for Radioresistant Tumors Recruiting NCT02838602 Not Applicable
42 A Study of IMRT in Primary Bone and Soft Tissue Sarcoma Recruiting NCT02520128 Not Applicable
43 Effect of High Doses of Radiation on Bone Structure and Metabolism Recruiting NCT02323295 Not Applicable
44 High Dose Intensity Modulated Proton Radiation Treatment +/- Surgical Resection of Sarcomas of the Spine, Sacrum and Base of Skull Active, not recruiting NCT01346124 Not Applicable
45 Stereotactic Body Radiotherapy for Spine Tumors Active, not recruiting NCT01347307 Not Applicable
46 Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma Active, not recruiting NCT00919269
47 Innovative SolutIons for DosimEtry in Hadrontherapy (INSIDE) Not yet recruiting NCT03662373

Search NIH Clinical Center for Chordoma

Cochrane evidence based reviews: chordoma

Genetic Tests for Chordoma

Genetic tests related to Chordoma:

# Genetic test Affiliating Genes
1 Chordoma 29

Anatomical Context for Chordoma

MalaCards organs/tissues related to Chordoma:

41
Bone, Lung, Brain, Liver, Pituitary, Thyroid, Breast

Publications for Chordoma

Articles related to Chordoma:

(show top 50) (show all 1117)
# Title Authors Year
1
Letter to the Editor. Brachyury as prognostic biomarker in chordoma. ( 29701545 )
2018
2
SNF5 as a prognostic factor in skull base chordoma. ( 29222701 )
2018
3
Computed tomography and magnetic resonance imaging features of cervical chordoma. ( 29963156 )
2018
4
Clinical experience of intracranial chordoma - A systematic review and meta-analysis of the literature. ( 29724651 )
2018
5
In Reply: Cranial Chordoma: A New Preoperative Grading System. ( 29672767 )
2018
6
Letter: Cranial Chordoma: A New Preoperative Grading System. ( 29672759 )
2018
7
A historical recount of chordoma. ( 29393831 )
2018
8
Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014. ( 29625617 )
2018
9
Humeral metastasis of sacrococcygeal chordoma detected by fluorine-18 fluorodeoxyglucose positron emission tomography-computed tomography: A case report. ( 29904494 )
2018
10
Clinicopathologic implications of CD8<sup>+</sup>/Foxp3<sup>+</sup>ratio and miR-574-3p/PD-L1 axis in spinal chordoma patients. ( 29051990 )
2018
11
Intradural cervical chordoma with diffuse spinal leptomeningeal spread: case report and review of the literature. ( 29313091 )
2018
12
Chordoma ( 28613596 )
2018
13
Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma. ( 29850323 )
2018
14
Extended endoscopic endonasal surgery for clival chordoma and chondrosarcoma: Our experience in 14 cases. ( 29691145 )
2018
15
Prospective validation of a molecular prognostication panel for clival chordoma. ( 29905508 )
2018
16
Radiofrequency ablation in gross total excision of cervical chordoma: ideas and technique. ( 29915886 )
2018
17
Analysis of clinical factors and PDGFR-I^ in predicting prognosis of patients with clival chordoma. ( 29303447 )
2018
18
Clival chordoma with drop metastases. ( 29875988 )
2018
19
A giant lumbar chordoma: A case report. ( 29924012 )
2018
20
Role of the Apparent Diffusion Coefficient as a Predictor of Tumor Progression in Patients with Chordoma. ( 29724767 )
2018
21
The Sellar Tumor: Metastasis or Chordoma? ( 29381634 )
2018
22
Cryosurgery in the excision of a giant local recurrent sacral chordoma: a case report and literature review. ( 29372428 )
2018
23
Tumour Volume and Dose Influence Outcome after Surgery and High-dose Photon Radiotherapy for Chordoma and Chondrosarcoma of the Skull Base and Spine. ( 29402600 )
2018
24
miR-16-5p inhibits chordoma cell proliferation, invasion and metastasis by targeting Smad3. ( 29880900 )
2018
25
The embryonic transcription factor Brachyury confers chordoma chemoresistance via upregulating CA9. ( 29636883 )
2018
26
Characterization of the endolysosomal system in human chordoma cell lines: is there a role of lysosomes in chemoresistance of this rare bone tumor? ( 29725750 )
2018
27
Sacral chordoma : A review of literature. ( 29881220 )
2018
28
Cardiac Metastasis from Clivus Chordoma. ( 29454123 )
2018
29
Osteosarcoma, Chondrosarcoma, and Chordoma. ( 29220289 )
2018
30
Clival Chordoma With Brainstem Invasion. ( 29325600 )
2018
31
State-of-the-Art Imaging in Human Chordoma of the Skull Base. ( 29629241 )
2018
32
Clival Chondroid Chordoma: A Case Report and Review of the Literature. ( 30519520 )
2018
33
In Reply: Cranial Chordoma: A New Preoperative Grading System. ( 29566169 )
2018
34
Letter: Cranial Chordoma: A New Preoperative Grading System. ( 29566218 )
2018
35
Comparison of the Effectiveness of Radiotherapy with Photons and Particles for Chordoma After Surgery: A Meta-Analysis. ( 29879512 )
2018
36
Clinical Impact of the Immune Microenvironment in Spinal Chordoma: Immunoscore as an Independent Favorable Prognostic Factor. ( 30032257 )
2018
37
Surgical management of chordoma: A systematic review. ( 30048230 )
2018
38
Four-Level Vertebrectomy for En Bloc Resection of a Cervical Chordoma. ( 30059783 )
2018
39
Cost-Effectiveness of Carbon Ion Radiation Therapy for Skull Base Chordoma Utilizing Long-Term (10-Year) Outcome Data. ( 30061259 )
2018
40
Analysis of variants at LGALS3 single nucleotide polymorphism loci in skull base chordoma. ( 30061951 )
2018
41
Sacral chordoma: clinical experience of a series of 11 patients over 18 years. ( 30066091 )
2018
42
A Phase 1 Study of Nilotinib Plus Radiation in High-Risk Chordoma. ( 30077789 )
2018
43
Development of Machine Learning Algorithms for Prediction of 5-Year Spinal Chordoma Survival. ( 30096498 )
2018
44
The timing of fusion surgery for clival chordoma with occipito-cervical joint instability: before or after tumor resection? ( 30116987 )
2018
45
Chordoma: a case series and review of the literature. ( 30145982 )
2018
46
Supratotal Resection of Residual Clival Chordoma With Combined Endoscopic Endonasal and Contralateral Transmaxillary Approaches: 2-Dimensional Operative Video. ( 30189024 )
2018
47
Clival chordoma: long-term clinical outcome in a single center. ( 30200131 )
2018
48
Surgical Treatment of Sacral Chordoma: En Bloc Resection with Negative Margins is a Determinant of the Long-Term Outcome. ( 30204925 )
2018
49
A Staged Strategy for Craniocervical Junction Chordoma with Combination of Endoscopic Endonasal Approach and Far Lateral Approach with Endoscopic Assistance: Case Report. ( 30210992 )
2018
50
Imatinib and everolimus in patients with progressing advanced chordoma: A phase 2 clinical study. ( 30216418 )
2018

Variations for Chordoma

Cosmic variations for Chordoma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM6976986 SETD2 bone,sacrum,chordoma,NS c.3341C>T p.A1114V 3:47103413-47103413 20
2 COSM1307 KIT bone,sacrum,chordoma,NS c.2394C>T p.I798I 4:54733102-54733102 20
3 COSM21983 KIT bone,sacrum,chordoma,NS c.1638A>G p.K546K 4:54727315-54727315 20
4 COSM6976985 bone,sacrum,chordoma,NS c.4850C>T p.A1617V 3:47103413-47103413 20

Copy number variations for Chordoma from CNVD:

7 (show top 50) (show all 470)
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 13362 10 1 135374737 Loss PTEN Chordoma
2 13372 17 1 22200000 Loss NF1 Chordoma
3 13373 17 1 22200000 Loss P53 Chordoma
4 13795 11 1 51400000 Loss WT1 Chordoma
5 13810 19 1 63811651 Gain BAX Chordoma
6 13811 19 1 63811651 Gain TGFB1 Chordoma
7 13825 18 1 76117153 Loss SMAD4 Chordoma
8 17403 1 142600000 180300000 Gain Chordoma
9 21008 1 15600000 106368585 Loss Chordoma
10 21654 13 16000000 114142980 Loss BRCA2 Chordoma
11 21655 13 16000000 114142980 Loss RB Chordoma
12 25739 1 193800000 243700000 Gain Chordoma
13 28392 17 22200000 78774742 Gain HER2 Chordoma
14 29289 1 2300000 124300000 Loss RUNX3 Chordoma
15 31074 1 25226001 25291501 Loss RUNX3 Chordoma
16 31348 1 26979575 26979875 Gain Chordoma
17 31733 1 30245671 30246671 Gain Chordoma
18 31901 1 31899334 31900334 Loss Chordoma
19 31908 1 31940315 31941315 Gain Chordoma
20 32031 1 32719745 32720045 Gain Chordoma
21 32032 1 32720036 32721036 Loss Chordoma
22 32207 1 34451346 34452346 Gain Chordoma
23 32675 1 38399550 38400550 Gain Chordoma
24 32770 1 39264122 39265122 Gain Chordoma
25 33966 1 50992002 50993002 Gain Chordoma
26 35065 11 59700000 74900000 Gain MEN1 Chordoma
27 36036 1 70901720 70902020 Loss Chordoma
28 36038 1 70998381 70998681 Loss Chordoma
29 36075 1 71137728 71138728 Gain Chordoma
30 48312 11 1 12700000 Loss Chordoma
31 49581 11 108093558 108239826 Loss ATM Chordoma
32 52925 11 21700000 48800000 Loss Chordoma
33 56520 11 59900000 68400000 Loss Chordoma
34 63736 12 120700000 125900000 Loss Chordoma
35 65974 12 23685230 24715380 Gain SOX5 Chordoma
36 66550 12 30700000 35800000 Loss Chordoma
37 67677 12 4382901 4414522 Gain CCND2 Chordoma
38 67847 12 4543308 4554780 Gain Chordoma
39 68279 12 48366747 48398285 Gain COL2A1 Chordoma
40 74311 13 103858404 103859404 Loss Chordoma
41 81414 14 102320080 102321080 Gain Chordoma
42 81835 14 104931320 104931620 Gain Chordoma
43 81836 14 104931398 104932398 Loss Chordoma
44 86968 14 67892033 67893033 Loss Chordoma
45 86969 14 67894685 67895685 Gain Chordoma
46 86990 14 68271232 68272232 Gain Chordoma
47 87006 14 68408332 68409332 Gain Chordoma
48 87676 14 75864127 75864427 Loss Chordoma
49 87678 14 75887005 75888005 Gain Chordoma
50 87692 14 76184263 76185263 Loss Chordoma

Expression for Chordoma

Search GEO for disease gene expression data for Chordoma.

Pathways for Chordoma

Pathways related to Chordoma according to GeneCards Suite gene sharing:

(show all 24)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.92 CDH1 CEACAM3 CTSK EGFR LGALS3 MET
2
Show member pathways
13.74 CDH1 CDKN2A DES EGFR GFAP MET
3
Show member pathways
12.76 CDH1 CDKN2A EGFR PDGFRA PTEN
4 12.71 CDH1 CDKN2A EGFR MET PDGFRA PTEN
5
Show member pathways
12.58 CDH1 CDKN2A EGFR MET PDGFRA PTEN
6
Show member pathways
12.55 CDH1 CDKN2A EGFR MET PTEN SMARCB1
7 12.12 CDH1 DES KRT19 KRT7 MUC1 VIM
8
Show member pathways
12.08 EGFR MET PDGFRA PTEN
9 12.02 EGFR MET PDGFRA PTEN
10 11.95 KRT19 MET TBXT VIM
11 11.91 CDKN2A EGFR MET PDGFRA PTEN VIM
12 11.87 EGFR GFAP LGALS3 VIM
13 11.8 CDKN2A EGFR PDGFRA PTEN
14
Show member pathways
11.75 CDH1 EGFR MET
15 11.63 CDH1 CDKN2A EGFR
16 11.62 CDH1 EGFR MET
17 11.57 GFAP PDGFRA S100B VIM
18 11.44 CDKN2A EGFR PDGFRA
19 11.44 EGFR MET PDGFRA PTEN
20 11.31 CDH1 EGFR MET
21 11.14 CDH1 EGFR MET
22
Show member pathways
11.05 DES GFAP KRT19 KRT7 VIM
23 10.72 LGALS3 S100B
24 10.7 CDH1 DES EGFR GFAP MET PDGFRA

GO Terms for Chordoma

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

# Name GO ID Score Top Affiliating Genes
1 cytoplasm GO:0005737 9.86 CDH1 CDKN2A CTSK DES EGFR GFAP
2 extracellular region GO:0005576 9.81 CDH1 CTSK EGFR LGALS3 MET MUC1
3 intermediate filament GO:0005882 9.02 DES GFAP KRT19 KRT7 VIM

Biological processes related to Chordoma according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.89 EGFR PDGFRA PTEN S100B TBXT
2 viral process GO:0016032 9.85 KRT19 KRT7 PDGFRA SMARCB1 VIM
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.78 EGFR MET PDGFRA PTEN
4 learning or memory GO:0007611 9.5 EGFR PTEN S100B
5 Bergmann glial cell differentiation GO:0060020 9.43 GFAP VIM
6 entry of bacterium into host cell GO:0035635 9.4 CDH1 MET
7 obsolete negative regulation of cyclin-dependent protein serine/threonine kinase activity involved in G1/S transition of mitotic cell cycle GO:0031658 9.37 CDKN2A PTEN
8 long-term synaptic potentiation GO:0060291 9.33 GFAP PTEN S100B
9 positive regulation of histone H4 acetylation GO:0090240 9.26 MUC1 SMARCB1
10 intermediate filament-based process GO:0045103 8.96 GFAP VIM
11 intermediate filament organization GO:0045109 8.8 DES GFAP VIM

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

# Name GO ID Score Top Affiliating Genes
1 protein phosphatase binding GO:0019903 9.54 EGFR LGALS3 MET
2 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 9.5 EGFR MET PDGFRA
3 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.33 EGFR MET PDGFRA
4 identical protein binding GO:0042802 9.28 CDH1 DES EGFR GFAP MET PTEN
5 structural constituent of cytoskeleton GO:0005200 9.26 DES GFAP KRT19 VIM
6 protein binding GO:0005515 10.19 CDH1 CDKN2A CTSK DES EGFR GFAP

Sources for Chordoma

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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