MCID: CHR072
MIFTS: 61

Chordoma

Categories: Rare diseases, Cancer diseases, Endocrine diseases

Aliases & Classifications for Chordoma

MalaCards integrated aliases for Chordoma:

Name: Chordoma 53 72 49 24 55 71 28 13 51 41 69
Chdm 53 24 71
Notochordal Sarcoma 24 55
Chordoma, Susceptibility to 53
Chordoepithelioma 24
Chordocarcinoma 24
Notochordoma 24

Characteristics:

Orphanet epidemiological data:

55
chordoma
Inheritance: Autosomal dominant,Not applicable; Prevalence: <1/1000000 (United States); Age of onset: Adult; Age of death: any age;

OMIM:

53
Inheritance:
autosomal dominant

Miscellaneous:
median age at diagnosis, 59 years


HPO:

31
chordoma:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 55  
Rare endocrine diseases


External Ids:

OMIM 53 215400
Orphanet 55 ORPHA178
MESH via Orphanet 42 D002817
UMLS via Orphanet 70 C0008487
ICD10 via Orphanet 33 C76.7
MedGen 39 C0008487
MeSH 41 D002817
SNOMED-CT via HPO 65 263681008 50007008
UMLS 69 C0008487

Summaries for Chordoma

NIH Rare Diseases : 49 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. Last updated: 8/11/2017

MalaCards based summary : Chordoma, also known as chdm, is related to spinal chordoma and tuberous sclerosis, and has symptoms including abnormality of the vertebral column and abnormality of the head. An important gene associated with Chordoma is CHDM (Chordoma), and among its related pathways/superpathways are Endometrial cancer and Pathways in cancer. The drugs Sunitinib and Palbociclib have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and liver, and related phenotypes are Decreased cell migration and behavior/neurological

Genetics Home Reference : 24 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 : 53 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 : 71 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 : 72 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 281)
# Related Disease Score Top Affiliating Genes
1 spinal chordoma 33.9 EGFR MET S100B
2 tuberous sclerosis 30.5 GFAP S100B VIM
3 non-functioning pituitary adenoma 30.2 MUC1 S100B
4 rhabdoid meningioma 30.1 GFAP VIM
5 giant cell tumor 30.1 DES MUC1 VIM
6 myoepithelioma 30.1 GFAP S100B VIM
7 chordoid meningioma 29.9 GFAP MUC1 S100B VIM
8 hemangioblastoma 29.9 GFAP MUC1 S100B
9 pleomorphic adenoma 29.9 GFAP MUC1 S100B VIM
10 alveolar soft part sarcoma 29.9 DES MET VIM
11 hemangiopericytoma, malignant 29.9 DES MUC1 S100B VIM
12 epithelioid sarcoma 29.8 KRT7 MUC1 VIM
13 parachordoma 29.8 DES GFAP KRT7 MUC1 VIM
14 adenocarcinoma 29.7 CDKN2A EGFR MET MUC1
15 horner's syndrome 29.7 DES KRT7
16 ependymoma 29.6 EGFR GFAP MUC1 S100B VIM
17 meningioma, familial 29.5 GFAP MUC1 NCAM1 S100B VIM
18 myxopapillary ependymoma 29.5 GFAP MUC1 NCAM1 S100B VIM
19 renal cell carcinoma, nonpapillary 29.3 EGFR KRT7 MET MUC1 VIM
20 neurofibroma 29.2 CDKN2A MUC1 PDGFRA S100B
21 astrocytoma 29.1 CDKN2A EGFR GFAP S100B VIM
22 small cell carcinoma 28.7 EGFR KRT7 NCAM1 PDGFRA
23 chondroid chordoma 12.4
24 clivus chordoma 12.3
25 clivus chondroid chordoma 12.3
26 sacrum chordoma 12.2
27 localized hypertrophic neuropathy 10.6 MUC1 S100B
28 sclerosing perineurioma 10.6 MUC1 S100B
29 hydromyelia 10.5 S100B VIM
30 papillary tumor of the pineal region 10.5 MUC1 VIM
31 bednar tumor 10.5 S100B VIM
32 skin glomus tumor 10.5 DES VIM
33 infantile digital fibromatosis 10.5 DES VIM
34 primary hepatic neuroendocrine carcinoma 10.5 S100B VIM
35 lymphangiomatosis 10.5 DES VIM
36 epithelioid malignant peripheral nerve sheath tumor 10.5 MUC1 S100B
37 spindle cell thymoma 10.5 DES MUC1
38 angiolipoma 10.5 DES VIM
39 gliomatosis peritonei 10.5 DES GFAP
40 breast scirrhous carcinoma 10.5 EGFR MUC1
41 spindle cell lipoma 10.5 DES VIM
42 gemistocytic astrocytoma 10.5 GFAP S100B
43 large cell medulloblastoma 10.5 MET VIM
44 malignant triton tumor 10.5 DES S100B
45 epulis 10.5 DES VIM
46 cervicitis 10.5
47 adenofibroma 10.5 MUC1 VIM
48 epithelioid leiomyosarcoma 10.4 DES VIM
49 aggressive digital papillary adenocarcinoma 10.4 MUC1 S100B
50 dysembryoplastic neuroepithelial tumor 10.4 GFAP S100B

Graphical network of the top 20 diseases related to Chordoma:



Diseases related to Chordoma

Symptoms & Phenotypes for Chordoma

Symptoms via clinical synopsis from OMIM:

53
Skeletal Skull:
sphenooccipital chordoma
nasopharyngeal chordoma

Skeletal Spine:
vertebral chordoma
sacrococcygeal chordoma


Clinical features from OMIM:

215400

Human phenotypes related to Chordoma:

31
# Description HPO Frequency HPO Source Accession
1 abnormality of the vertebral column 31 HP:0000925
2 abnormality of the head 31 HP:0000234
3 chordoma 31 HP:0010762

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

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased cell migration GR00055-A-1 9.1 EGFR JUP MET MUC1 NCAM1 VIM

MGI Mouse Phenotypes related to Chordoma:

43 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.24 CDKN2A DES GFAP JUP LGALS3 MET
2 cellular MP:0005384 10.2 JUP KRT7 LGALS3 MET NCAM1 PDGFRA
3 cardiovascular system MP:0005385 10.18 CDKN2A DES EGFR GFAP JUP LGALS3
4 homeostasis/metabolism MP:0005376 10.15 CDKN2A DES EGFR GFAP JUP KRT7
5 mortality/aging MP:0010768 10 MET NCAM1 PDGFRA TBXT VIM CDKN2A
6 digestive/alimentary MP:0005381 9.98 CDKN2A EGFR GFAP LGALS3 MET PDGFRA
7 embryo MP:0005380 9.95 CDKN2A EGFR JUP MET PDGFRA TBXT
8 integument MP:0010771 9.87 CDKN2A EGFR JUP LGALS3 PDGFRA S100B
9 muscle MP:0005369 9.76 CDKN2A DES EGFR GFAP JUP MET
10 nervous system MP:0003631 9.65 LGALS3 MET NCAM1 PDGFRA TBXT VIM
11 respiratory system MP:0005388 9.17 CDKN2A EGFR JUP LGALS3 MET PDGFRA

Drugs & Therapeutics for Chordoma

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

(show all 49)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
2
Palbociclib Approved, Investigational Phase 2 571190-30-2 5330286 11431660 5005498
3
nivolumab Approved Phase 2,Phase 1 946414-94-4
4
Trioxsalen Approved Phase 2,Phase 1 3902-71-4 5585
5
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
6 Imatinib Mesylate Phase 2,Phase 1 123596
7 Protein Kinase Inhibitors Phase 2,Phase 1
8 Angiogenesis Inhibitors Phase 2
9 Angiogenesis Modulating Agents Phase 2
10 Immunosuppressive Agents Phase 2,Phase 1
11 Vaccines Phase 2
12 Antibodies Phase 2,Phase 1
13 Antibodies, Monoclonal Phase 2,Phase 1
14 Immunoglobulins Phase 2,Phase 1
15 Dermatologic Agents Phase 2,Phase 1
16 Dihematoporphyrin Ether Phase 2,Phase 1
17 Ether Phase 2,Phase 1
18 Hematoporphyrin Derivative Phase 2,Phase 1
19 Hematoporphyrins Phase 2,Phase 1
20 Photosensitizing Agents Phase 2,Phase 1
21
Cobalt Phase 2 7440-48-4 104729
22 Pharmaceutical Solutions Phase 2
23 Brewer's Yeast Nutraceutical Phase 2
24 lysine Nutraceutical Phase 2,Phase 1
25
Panobinostat Approved, Investigational Phase 1 404950-80-7 6918837
26
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
27
Amifostine Approved, Investigational Phase 1 20537-88-6 2141
28
Busulfan Approved, Investigational Phase 1 55-98-1 2478
29
Lenograstim Approved, Investigational Phase 1 135968-09-1
30
Melphalan Approved Phase 1 148-82-3 460612 4053
31 Thiotepa Approved, Investigational Phase 1 52-24-4 5453
32 Histone Deacetylase Inhibitors Phase 1
33 Mitogens Phase 1
34 Adjuvants, Immunologic Phase 1
35 Protective Agents Phase 1
36 Alkylating Agents Phase 1
37 Antirheumatic Agents Phase 1
38 Liver Extracts Phase 1
39 Radiation-Protective Agents Phase 1
40
Hyaluronic acid Approved, Vet_approved 9004-61-9 53477741 24759
41
Povidone Approved 9003-39-8
42
Menthol Approved 2216-51-5 16666
43 Misonidazole Investigational 13551-87-6
44 Blood Substitutes
45 Plasma Substitutes
46 Viscosupplements
47 Anti-Infective Agents
48 Antiparasitic Agents
49 Antiprotozoal Agents

Interventional clinical trials:

(show all 42)

# Name Status NCT ID Phase Drugs
1 Stereotactic Body Radiotherapy for Head and Neck Tumors Active, not recruiting 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 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
7 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
8 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
9 CDK4/6 Inhibition in Locally Advanced/Metastatic Chordoma Recruiting NCT03110744 Phase 2 Palbociclib
10 Ion Irradiation of Sacrococcygeal Chordoma Recruiting NCT01811394 Phase 2
11 The National Cancer Institute Opens A Randomized, Double-Blind, Phase 2 Trial of GI-6301 (Yeast-Brachyury Vaccine) Versus Placebo in Combination With Standard of Care Definitive Radiotherapy in Locally Advanced, Unresectable, Chordoma Recruiting NCT02383498 Phase 2
12 Immune Checkpoint Inhibitor Nivolumab in People With Select Rare CNS Cancers Recruiting NCT03173950 Phase 2 Nivolumab
13 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
14 A Phase II Study Evaluating Efficacy and Safety of Regorafenib in Patients With Metastatic Bone Sarcomas Recruiting NCT02389244 Phase 2 Regorafenib;Placebo
15 Nivolumab and Ipilimumab in Treating Patients With Rare Tumors Recruiting NCT02834013 Phase 2
16 Proton Beam Therapy for Chordoma Patients Active, not recruiting NCT00496119 Phase 2
17 Trial of Dasatinib in Advanced Sarcomas Active, not recruiting NCT00464620 Phase 2 Dasatinib
18 Photodynamic Therapy (PDT) For Recurrent High Grade Gliomas Active, not recruiting NCT01966809 Phase 2 Photofrin photodynamic therapy.
19 Afatinib in Locally Advanced and Metastatic Chordoma Not yet recruiting NCT03083678 Phase 2 Afatinib
20 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
21 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)
22 Nivolumab With or Without Stereotactic Radiosurgery in Treating Patients With Recurrent, Advanced, or Metastatic Chordoma Recruiting NCT02989636 Phase 1
23 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Recruiting NCT02601937 Phase 1 Tazemetostat
24 HSV1716 in Patients With Non-Central Nervous System (Non-CNS) Solid Tumors Recruiting NCT00931931 Phase 1
25 Photodynamic Therapy (PDT) for Recurrent Pediatric Brain Tumors Recruiting NCT01682746 Phase 1 Photofrin (porfimer sodium) & photodynamic therapy.
26 Nilotinib With Radiation for High Risk Chordoma Active, not recruiting NCT01407198 Phase 1 Nilotinib
27 Safety Study of Intratumoral Injection of Clostridium Novyi-NT Spores to Treat Patients With Solid Tumors That Have Not Responded to Standard Therapies Active, not recruiting NCT01924689 Phase 1
28 Amifostine to Protect From Side Effects of PSCT in Treating Patients With Solid Tumors Terminated NCT00003926 Phase 1 amifostine trihydrate;busulfan;filgrastim;melphalan;thiotepa
29 Polyvinylpyrrolidone-Sodium Hyaluronate Gel in Reducing Pain From Oral Mucositis in Young Patients With Cancer Unknown status NCT00349024 polyvinylpyrrolidone-sodium hyaluronate gel
30 Chordoma Family Study Completed NCT00410670
31 Genetic Aspects of Chordoma: A Collaboration With SEER Registries to Identify Chordoma Families Completed NCT00341627
32 Hypoxia-positron Emission Tomography (PET) and Intensity Modulated Proton Therapy (IMPT) Dose Painting in Patients With Chordomas Completed NCT00713037
33 Proton Therapy for Chordomas and/or Chondrosarcomas Completed NCT00797602
34 Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas Completed NCT01567046
35 Sacral Chordoma: Surgery Versus Definitive Radiation Therapy in Primary Localized Disease Recruiting NCT02986516
36 Genetic Clues to Chordoma Etiology: A Protocol to Identify Sporadic Chordoma Patients for Studies of Cancer-Susceptibility Genes Recruiting NCT01200680
37 A Study of IMRT in Primary Bone and Soft Tissue Sarcoma Recruiting NCT02520128
38 Effect of High Doses of Radiation on Bone Structure and Metabolism Recruiting NCT02323295
39 High Dose Intensity Modulated Proton Radiation Treatment +/- Surgical Resection of Sarcomas of the Spine, Sacrum and Base of Skull Active, not recruiting NCT01346124
40 Stereotactic Body Radiotherapy for Spine Tumors Active, not recruiting NCT01347307
41 Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma Active, not recruiting NCT00919269
42 Randomized Carbon Ions vs Standard Radiotherapy for Radioresistant Tumors Not yet recruiting NCT02838602

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 28

Anatomical Context for Chordoma

MalaCards organs/tissues related to Chordoma:

38
Bone, Lung, Liver, Brain, Bone Marrow, Pituitary, Thyroid

Publications for Chordoma

Articles related to Chordoma:

(show top 50) (show all 832)
# Title Authors Year
1
Osteosarcoma, Chondrosarcoma, and Chordoma. ( 29220289 )
2018
2
Clival Chordoma With Brainstem Invasion. ( 29325600 )
2018
3
The Sellar Tumor: Metastasis or Chordoma? ( 29381634 )
2018
4
Analysis of clinical factors and PDGFR-I^ in predicting prognosis of patients with clival chordoma. ( 29303447 )
2018
5
Intradural cervical chordoma with diffuse spinal leptomeningeal spread: case report and review of the literature. ( 29313091 )
2018
6
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
7
Clinicopathologic implications of CD8<sup>+</sup>/Foxp3<sup>+</sup>ratio and miR-574-3p/PD-L1 axis in spinal chordoma patients. ( 29051990 )
2018
8
SNF5 as a prognostic factor in skull base chordoma. ( 29222701 )
2018
9
A historical recount of chordoma. ( 29393831 )
2018
10
Cryosurgery in the excision of a giant local recurrent sacral chordoma: a case report and literature review. ( 29372428 )
2018
11
Removal of a low clival chordoma in a teenager by dorsolateral suboccipital transcondylar approach. ( 28761544 )
2017
12
First report of clinical responses to immunotherapy in 3 relapsing cases of chordoma after failure of standard therapies. ( 28919999 )
2017
13
T1-Weighted Dynamic Contrast-Enhanced MR Perfusion Imaging Characterizes Tumor Response to Radiation Therapy in Chordoma. ( 28912284 )
2017
14
Clival chordoma: a single-centre outcome analysis. ( 28478512 )
2017
15
Role of intraoperative radiotherapy in the treatment of sacral chordoma. ( 28882523 )
2017
16
Letter to the Editor concerning "Surgical treatment of sacral chordoma: survival and prognostic factors" by C. Ruosi et al. (Eur Spine J; 2015; 24(Suppl 7):S912-S917. ( 27757683 )
2017
17
Prognostic Factors in Patients With Spinal Chordoma: An Integrative Analysis of 682 Patients. ( 29029249 )
2017
18
Iatrogenic seeding of skull base chordoma following endoscopic endonasal surgery. ( 29271711 )
2017
19
Chordoma Occurs in Young Children With Tuberous Sclerosis. ( 28498973 )
2017
20
Long non-coding RNA LOC554202 modulates chordoma cell proliferation and invasion by recruiting EZH2 and regulating miR-31 expression. ( 28963737 )
2017
21
Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. AA retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression FranAsaise (ANOCEF). ( 28478340 )
2017
22
Afatinib is a new therapeutic approach in chordoma with a unique ability to target EGFR and Brachyury. ( 29237806 )
2017
23
On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma. ( 28409046 )
2017
24
Prognostic Significance of Resection Degree in Skull Base Chordoma: A Systematic Review and Meta-Analysis. ( 28437876 )
2017
25
Updated Outcome and Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High-Dose Photon/Proton Radiation Therapy. ( 27986348 )
2017
26
EGFR Inhibition in a Pretreated Sacral Chordoma: A Role for Erlotinib? Case Report and a Brief Review of Literature. ( 28775967 )
2017
27
In Reply: Factors Predicting Recurrence After Resection of Clival Chordoma Using Variable Surgical Approaches and Radiation Modalities. ( 28379552 )
2017
28
Prognostic Factors and Survival Outcomes in Patients with Chordoma in the United States: A Population-Based Analysis. ( 28457925 )
2017
29
Whole-Body MRI Virtual Autopsy Using Diffusion-weighted Imaging With Background Suppression (DWIBS) at 3 T in a Child Succumbing to Chordoma. ( 28060123 )
2017
30
CDK4 expression in chordoma: A potential therapeutic target. ( 29194728 )
2017
31
In Reply to "Prognostic Significance of Resection Degree inA Skull Base Chordoma: A Systematic Review and Meta-Analysis". ( 28437877 )
2017
32
Differences in Dural Penetration of Clival Chordomas Are Associated with Different Prognosis and Expression of Platelet-Derived Growth Factor Receptor-I^. ( 27506406 )
2017
33
Large chordoma of the sacrum. ( 28765485 )
2017
34
Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients. ( 28059654 )
2017
35
MR Imaging Grading System for Skull Base Chordoma. ( 28428207 )
2017
36
iASPP overexpression is associated with clinical outcome in spinal chordoma and influences cellular proliferation, invasion, and sensitivity to cisplatin<i>in vitro</i>. ( 28978123 )
2017
37
Impact of early access to multidisciplinary care on treatment outcomes in patients with skull base chordoma. ( 29270681 )
2017
38
Phase 2 study of dasatinib in patients with alveolar soft part sarcoma, chondrosarcoma, chordoma, epithelioid sarcoma, or solitary fibrous tumor. ( 27696380 )
2017
39
Neuronavigation-guided endoscopic endonasal excision of schwannoma-like chordoma of the Meckel's cave: A case report. ( 28758183 )
2017
40
Multicentric Chordoma : An Uncommon and Incompletely Understood Presentation. ( 28766007 )
2017
41
An unusual case of oropharyngeal chordoma: A case report and literature review. ( 29310398 )
2017
42
Recurrent chordoma with orbital and eyelid invasion. ( 28357494 )
2017
43
MRI evaluation of sacral chordoma treated with carbon ion radiotherapy alone. ( 29279241 )
2017
44
Genetic aberrations and molecular biology of skull base chordoma and chondrosarcoma. ( 28432450 )
2017
45
Letter to the Editor: Influence of age on survival outcomes in patients with spinal chordoma. ( 28291405 )
2017
46
EM proves invaluable in the confirmation of chordoma in the sacral mass of a middle-aged man. ( 28524812 )
2017
47
Intradural Chordoma of Cerebellopontine Angle: Case Report and Review. ( 28866065 )
2017
48
A Diagnostic Pitfall: Atypical Teratoid Rhabdoid Tumor Versus Dedifferentiated/Poorly Differentiated Chordoma: Analysis of a Mono-institutional Series. ( 28777153 )
2017
49
Establishment and genomic characterization of the new chordoma cell line Chor-IN-1. ( 28835717 )
2017
50
Spinal stereotactic body radiotherapy following intralesional curettage with separation surgery for initial or salvage chordoma treatment. ( 28041314 )
2017

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 Conf
1 COSM1307 KIT bone,sacrum,chordoma,NS c.2394C>T p.I798I 20
2 COSM21983 KIT bone,sacrum,chordoma,NS c.1638A>G p.K546K 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 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.85 CDKN2A EGFR JUP MET PDGFRA
2 12.63 CDKN2A EGFR JUP MET PDGFRA
3 12.25 DES JUP KRT7 MUC1 VIM
4 12.2 CDKN2A EGFR MET PDGFRA VIM
5 11.83 MET NCAM1 TBXT VIM
6 11.82 JUP MUC1 NCAM1
7 11.77 CDKN2A EGFR PDGFRA
8
Show member pathways
11.75 EGFR GFAP S100B
9 11.64 EGFR GFAP LGALS3 VIM
10 11.48 EGFR MET PDGFRA
11 11.38 NCAM1 PDGFRA TBXT
12 11.34 CDKN2A EGFR PDGFRA
13
Show member pathways
11.29 DES GFAP KRT7 VIM
14 11.2 LGALS3 MUC1 NCAM1
15 10.92 GFAP NCAM1 PDGFRA S100B VIM
16 10.64 LGALS3 S100B
17 10.54 DES EGFR GFAP MET PDGFRA VIM

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 9.93 CDKN2A DES EGFR GFAP JUP KRT7
2 extracellular matrix GO:0031012 9.56 JUP LGALS3 NCAM1 VIM
3 intermediate filament GO:0005882 9.02 DES GFAP JUP KRT7 VIM
4 fascia adherens GO:0005916 8.96 DES JUP

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

# Name GO ID Score Top Affiliating Genes
1 MAPK cascade GO:0000165 9.56 EGFR MET NCAM1 PDGFRA
2 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.54 EGFR MET PDGFRA
3 phosphatidylinositol phosphorylation GO:0046854 9.5 EGFR MET PDGFRA
4 astrocyte development GO:0014002 9.4 GFAP VIM
5 Bergmann glial cell differentiation GO:0060020 9.16 GFAP VIM
6 intermediate filament-based process GO:0045103 8.96 GFAP VIM
7 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 identical protein binding GO:0042802 9.85 DES EGFR GFAP MET S100B VIM
2 structural constituent of cytoskeleton GO:0005200 9.58 DES GFAP VIM
3 structural molecule activity GO:0005198 9.56 GFAP JUP KRT7 VIM
4 protein phosphatase binding GO:0019903 9.5 EGFR JUP MET
5 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 9.33 EGFR MET PDGFRA
6 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.13 EGFR MET PDGFRA
7 Ras guanyl-nucleotide exchange factor activity GO:0005088 8.92 EGFR MET NCAM1 PDGFRA

Sources for Chordoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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