Raras
Buscar doenças, sintomas, genes...
Cordoma
ORPHA:178CID-10 · C76.7CID-11 · 2B5JOMIM 215400DOENÇA RARA

Cordomas são um tipo raro de câncer que se forma a partir de resquícios da notocorda (uma estrutura presente no desenvolvimento do embrião), na parte central do esqueleto, como na coluna vertebral e no crânio.

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Introdução

O que você precisa saber de cara

📋

Cordomas são um tipo raro de câncer que se forma a partir de resquícios da notocorda (uma estrutura presente no desenvolvimento do embrião), na parte central do esqueleto, como na coluna vertebral e no crânio.

Pesquisas ativas
23 ensaios
75 total registrados no ClinicalTrials.gov
Publicações científicas
4.744 artigos
Último publicado: 2026 Apr 13
Medicamentos
10 registrados
AFATINIB, PALBOCICLIB, NIVOLUMAB

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10 medicamentos registrados
Ver detalhes, fases e interações →
AFATINIBPALBOCICLIBNIVOLUMABIMATINIBCETUXIMABRELATLIMABPEMETREXEDNILOTINIBAVELUMABFILGRASTIM

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.06
United States
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C76.7
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Características mais comuns

94%prev.
Cordoma
Frequência: 15/16
50%prev.
Anomalia do desenvolvimento do giro frontal inferior
Frequência: 8/16
25%prev.
HP:0003596
Frequência: 4/16
19%prev.
Início juvenil
Frequência: 3/16
6%prev.
Astrocitoma
Frequência: 1/16
6%prev.
HP:0003584
Frequência: 1/16
7sintomas
Muito frequente (1)
Frequente (1)
Ocasional (4)
Sem dados (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 7 características clínicas mais associadas, ordenadas por frequência.

CordomaChordoma
Frequência: 15/1694%
Anomalia do desenvolvimento do giro frontal inferiorHP:0011462
Frequência: 8/1650%
HP:0003596
Frequência: 4/1625%
Início juvenilJuvenile onset
Frequência: 3/1619%
AstrocitomaAstrocytoma
Frequência: 1/166%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico4.744PubMed
Últimos 10 anos200publicações
Pico2025128 papers
Linha do tempo
2026Hoje · 2026🧪 1998Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

3 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.

PALB2Partner and localizer of BRCA2Major susceptibility factor inTolerante
FUNÇÃO

Plays a critical role in homologous recombination repair (HRR) through its ability to recruit BRCA2 and RAD51 to DNA breaks (PubMed:16793542, PubMed:19369211, PubMed:19423707, PubMed:22941656, PubMed:24141787, PubMed:28319063). Strongly stimulates the DNA strand-invasion activity of RAD51, stabilizes the nucleoprotein filament against a disruptive BRC3-BRC4 polypeptide and helps RAD51 to overcome the suppressive effect of replication protein A (RPA) (PubMed:20871615). Functionally cooperates wit

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (6)
Resolution of D-loop Structures through Holliday Junction IntermediatesHDR through Homologous Recombination (HRR)Resolution of D-loop Structures through Synthesis-Dependent Strand Annealing (SDSA)Homologous DNA Pairing and Strand ExchangeImpaired BRCA2 binding to PALB2
MECANISMO DE DOENÇA

Breast cancer

A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
13.6 TPM
Testículo
10.3 TPM
Fibroblastos
10.2 TPM
Cérebro - Hemisfério cerebelar
8.7 TPM
Cerebelo
8.4 TPM
OUTRAS DOENÇAS (9)
Fanconi anemia complementation group NPALB2-related cancer predispositionchordomahereditary breast carcinoma
HGNC:26144UniProt:Q86YC2
TBXTT-box transcription factor TMajor susceptibility factor inTolerante
FUNÇÃO

Involved in the transcriptional regulation of genes required for mesoderm formation and differentiation. Binds to a palindromic T site 5'-TTCACACCTAGGTGTGAA-3' DNA sequence and activates gene transcription when bound to such a site

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (6)
Epithelial-Mesenchymal Transition (EMT) during gastrulationGerm layer formation at gastrulationFormation of paraxial mesodermFormation of axial mesodermFormation of definitive endoderm
MECANISMO DE DOENÇA

Neural tube defects

Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy. Failure of neural tube closure can occur at any level of the embryonic axis. Common NTD forms include anencephaly, myelomeningocele and spina bifida, which result from the failure of fusion in the cranial and spinal region of the neural tube. NTDs have a multifactorial etiology encompassing both genetic and environmental components.

OUTRAS DOENÇAS (3)
sacral agenesis-abnormal ossification of the vertebral bodies-persistent notochordal canal syndromechordomaneural tube defects, susceptibility to
HGNC:11515UniProt:O15178
BRCA2Breast cancer type 2 susceptibility proteinMajor susceptibility factor inTolerante
FUNÇÃO

Involved in double-strand break repair and/or homologous recombination. Binds RAD51 and potentiates recombinational DNA repair by promoting assembly of RAD51 onto single-stranded DNA (ssDNA). Acts by targeting RAD51 to ssDNA over double-stranded DNA, enabling RAD51 to displace replication protein-A (RPA) from ssDNA and stabilizing RAD51-ssDNA filaments by blocking ATP hydrolysis. Part of a PALB2-scaffolded HR complex containing RAD51C and which is thought to play a role in DNA repair by HR. May

LOCALIZAÇÃO

NucleusCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (3)
Presynaptic phase of homologous DNA pairing and strand exchangeHDR through MMEJ (alt-NHEJ)Meiotic recombination
MECANISMO DE DOENÇA

Breast cancer

A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.

OUTRAS DOENÇAS (17)
Wilms tumor 1Fanconi anemia complementation group D1breast-ovarian cancer, familial, susceptibility to, 2BRCA2-related cancer predisposition
HGNC:1101UniProt:P51587

Medicamentos e terapias

AFATINIBPhase 2

Mecanismo: Receptor protein-tyrosine kinase erbB-2 inhibitor

PALBOCICLIBPhase 2

Mecanismo: CDK6/cyclin D1 inhibitor

NIVOLUMABPhase 2

Mecanismo: Programmed cell death protein 1 inhibitor

IMATINIBPhase 2

Mecanismo: Tyrosine-protein kinase ABL inhibitor

CETUXIMABPhase 2

Mecanismo: Epidermal growth factor receptor erbB1 inhibitor

RELATLIMABPhase 2

Mecanismo: Lymphocyte activation gene 3 protein inhibitor

PEMETREXEDPhase 1

Mecanismo: GAR transformylase inhibitor

NILOTINIBPhase 1

Mecanismo: Tyrosine-protein kinase ABL inhibitor

AVELUMABPhase 1

Mecanismo: Programmed cell death 1 ligand 1 other

FILGRASTIMPhase 1

Mecanismo: Granulocyte colony stimulating factor receptor agonist

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

24,454 variantes patogênicas registradas no ClinVar.

🧬 BRCA2: NM_000059.4(BRCA2):c.219del (p.Gln73fs) ()
🧬 BRCA2: NM_000059.4(BRCA2):c.849dup (p.Gly284fs) ()
🧬 BRCA2: NM_000059.4(BRCA2):c.6914del (p.Lys2305fs) ()
🧬 BRCA2: NM_000059.4(BRCA2):c.518_520delinsT (p.Gly173fs) ()
🧬 BRCA2: NM_000059.4(BRCA2):c.3226del (p.Ser1075_Val1076insTer) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 17 variantes classificadas pelo ClinVar.

12
3
2
Patogênica (70.6%)
VUS (17.6%)
Benigna (11.8%)
VARIANTES MAIS SIGNIFICATIVAS
BRCA2: NM_000059.4(BRCA2):c.2141A>C (p.Glu714Ala) [Conflicting classifications of pathogenicity]
PALB2: NM_024675.4(PALB2):c.398G>C (p.Ser133Thr) [Conflicting classifications of pathogenicity]
PALB2: NM_024675.4(PALB2):c.1600T>G (p.Ser534Ala) [Conflicting classifications of pathogenicity]
PALB2: NM_024675.4(PALB2):c.3494C>T (p.Ser1165Leu) [Conflicting classifications of pathogenicity]
PALB2: NM_024675.4(PALB2):c.2755G>A (p.Val919Ile) [Conflicting classifications of pathogenicity]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 214
1Fase 15
·Pré-clínico11
Medicamentos catalogadosEnsaios clínicos· 10 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Cordoma

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

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Outros ensaios clínicos

75 ensaios clínicos encontrados, 23 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥇Melhor nível de evidência: Revisão sistemática
Timeline de publicações
2.178 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 2.178

#1

Transdural Cisternotomy in the Extended Middle Fossa Approach.

Neurology India2026 Mar 01

Dolenc's pretemporal transcavernous approach and Kawase's s anterior transpetrosal approach are the two primary extradural middle cranial fossa approaches (EMFAs) to the central skullbase. The extended extradural phase of the surgery may potentially result in temporal lobe edema, compromising the surgical exposure and sometimes a postoperative temporal lobe venous infarction. The authors describe an alternative technique of cerebrospinal fluid (CSF) drainage called "transdural cisternotomy" in EMFA. In this, CSF is released from the chiasmatic cistern during the extradural stage of surgery. Two illustrative cases are shown, a case of petroclival meningioma and a petroclival intradural chordoma. Both patients underwent gross total tumor resection with good long-term outcome. Transdural cisternotomy was greatly beneficial in both the cases. Based on our experience with several such cases, the transdural cisternotomy described here is an easy, reproducible, and effective method of brain relaxation in EMFAs.

#2

Surgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.

Surgical neurology international2026

Sacral chordomas are rare malignant bone tumors that are locally aggressive and difficult to treat due to proximity to pelvic viscera, neurovascular structures, and the high risk of surgical morbidity. En bloc resection with negative margins remains the gold standard, but it is often not feasible in large or adherent tumors. Rectal perforation is a rare yet potentially catastrophic intraoperative complication that remains sparsely reported. We report the case of a 70-year-old man presenting with progressive back and gluteal pain, urinary difficulty, and bowel dysfunction. Magnetic resonance imaging revealed a giant sacrococcygeal chordoma (16.2 × 12.2 × 11.7 cm) compressing the rectum and sacral canal. Pre-operative arterial embolization of the bilateral internal iliac, median sacral, and feeding arteries was performed. During intralesional resection through a posterior approach, a rectal microperforation was detected, prompting intraoperative consultation with the general surgery team. The defect was repaired, and a trephine sigmoid colostomy was created. Tumor debulking was completed with an estimated blood loss of 300 mL. Histopathology confirmed chordoma. The patient recovered well, with a functional stoma and satisfactory wound healing. Managing giant sacrococcygeal chordomas utilizing the "gold standard," en bloc resection, may risk rectal perforation due to visceral adherence. Prompt intraoperative recognition and management of this adverse event are critical, along with a multidisciplinary approach to maximize patient safety.

#3

CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.

Cells2026 Feb 22

Chordomas are rare, highly morbid tumors arising from notochordal progenitor cells along the spinal axis, associated with severe neurological complications and high recurrence rates. Their resistance to conventional therapies and limited options beyond surgical resection and high-dose radiation underscore the urgent need for novel therapeutic targets. Publicly available preliminary RNA sequencing data from the Chordoma Foundation identified chitinase-3-like 1 (CHI3L1), a secreted glycoprotein implicated in immune checkpoint regulation and epithelial-mesenchymal transition (EMT), as a promising candidate for chordoma immunotherapy. Yet, the comprehensive function of CHI3L1 in chordoma immune response remains unclear. To evaluate its presence in chordoma, we employed RNA-based analyses alongside enzyme-linked immunosorbent assays (ELISA) on commercially available chordoma cell lines (JHC7, U-CH12, U-CH1, U-CH1-N) and human chordoma tumor specimens. Our results demonstrate elevated CHI3L1 expression in chordoma cells relative to notochordal precursors, with comparative analyses revealing higher CHI3L1 expression in the primary tumor relative to recurrent samples. These findings suggest the potential role of CHI3L1 in chordoma tumorigenesis, emphasizing its relevance as a biomarker and therapeutic target for primary tumors. Future studies are necessary to elucidate the mechanistic role of CHI3L1 in chordoma immune evasion and to explore targeted interventions that may improve patient outcomes in this aggressive cancer.

#4

CircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.

International journal of molecular sciences2026 Jan 19

Slow-growing and locally invasive, chordoma is a rare malignant bone tumor, with a reported annual worldwide incidence of 0.08 per 100,000 cases. It accounts for about 3 percent of all bone tumors and about 20 percent of primary spinal tumors. The incidence rates vary between countries and races, with white/Caucasian males in the 5th or 6th decade of life having a higher prevalence. Chordoma poses significant challenges because of its high recurrence rate and resistance to several standard treatment techniques. All cancers, including chordomas, have altered energy metabolism processes that contribute to their unchecked growth and survival. The significance of non-coding RNAs, particularly circular RNAs (circRNAs), as key regulators at the intersection of cellular metabolism and immune function has been highlighted by recent discoveries. By focusing on important glycolytic enzymes in tumor cells and altering metabolic reprogramming pathways, CircRNAs can influence cancer metabolic adaptability. Furthermore, via influencing immune cell functions as immunological checkpoint signaling and macrophage polarization, circRNAs influence immune evasion in the tumor microenvironment. These frequently happen via regulating important pathway signals, like PI3K/AKT/mTOR and NRF2, or by processes like miRNA sponging, creating a tumor microenvironment that is immunosuppressive and metabolically friendly. The translational pathway of circRNA-targeted therapeutics is promoted as a developing pharmacological entity in this review, which also highlights recent information on the control of circRNA-mediated immunometabolism in chordoma and examines numerous important molecular axes. There are promising opportunities to develop novel precision treatments for chordoma by considering circRNAs as dual regulators of immunological and metabolic networks.

#5

Bone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.

Clinical neurology and neurosurgery2026 Feb

Chordomas are rare malignant tumors arising from embryonic remnants of the notochord, most commonly affecting the axial skeleton. Although advances have been made in surgical resection and radiation therapy, systemic treatment options remain limited. Bone-modifying agents (BMAs), including zoledronic acid and denosumab, as well as multikinase inhibitors (MKIs) like lenvatinib and cabozantinib, have emerged as potential targeted therapies based on preclinical models. However, comparative real-world data evaluating their outcomes in chordoma patients is lacking. A retrospective cohort study was conducted using the TriNetX Research Network. Patients with chordoma (ICD-10-CM C41.0, C41.2, C41.4) were stratified into treatment groups based on receipt of zoledronic acid, denosumab, or multikinase inhibitors (MKIs). Propensity score matching was used to adjust for baseline confounders. Outcomes evaluated at 5 years post-diagnosis included all-cause mortality, pathologic fracture, spinal cord compression, and osteonecrosis. Risk ratios with 95 % confidence intervals were calculated. Compared to denosumab, zoledronic acid was associated with a higher 5-year mortality risk, but a lower osteonecrosis risk. Comparisons involving MKIs showed no difference in mortality. No pathologic fractures were reported across cohorts. Spinal cord compression was not different among treatments. This study highlights potential differences in survival and skeletal-related outcomes among chordoma patients treated with BMAs, specifically denosumab and MKIs. These preliminary findings underscore the need for prospective studies to better define optimal systemic therapies for this rare malignancy.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2.904 artigos no totalmostrando 197

2026

Advances in Stereotactic Navigation for Primary Spine and Pelvis Tumor Resection and Reconstruction: A Systematic Review.

HSS journal : the musculoskeletal journal of Hospital for Special Surgery
2026

Chopsticks mononostril approach with sphenoid sinus cranialization and septal mucosa suturing: a new minimally invasive concept and closure technique for endoscopic endonasal approaches.

Journal of neurosurgery
2026

Outcomes of the Translabyrinthine-Infralabyrinthine Approach for Resection of Jugular Foramen Chordomas.

The Journal of craniofacial surgery
2026

Hearing Outcomes in the Surgical Management of Petroclival Malignancies: Understanding the Impact of Surgical Strategy and Adjuvant Radiotherapy.

Operative neurosurgery (Hagerstown, Md.)
2026

Advances in Radiation Therapy for Primary Bone Malignancies.

Technology in cancer research &amp; treatment
2026

Transdural Cisternotomy in the Extended Middle Fossa Approach.

Neurology India
2026

Intraosseous Pseudoprogression vs. Progression and Recurrence of Clival Chordoma after Proton Radiation Therapy: Case Series.

Journal of neurological surgery. Part B, Skull base
2026

Individual and Community-Level Social Determinant Associations with Skull Base Chordoma and Chondrosarcoma Disparities in the United States.

Journal of neurological surgery. Part B, Skull base
2026

An insight into the pharmacoeconomics of carbon ion radiotherapy: a systematic review.

Radiation oncology journal
2026

Paraspinal Skip Lesion Encountered During the Resection of a Sacral Chordoma: A Case Report.

Cureus
2026

Oncolytic Adenoviral Infection of Chordoma Achieves Treatment Efficacy Associated with Immunologic Response and Tumor Microenvironmental Alteration.

Neuro-oncology
2026

Contemporary Management of Standard Chordoma Arising in the Mobile Spine and Sacrum.

The Journal of the American Academy of Orthopaedic Surgeons
2026

Surgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.

Surgical neurology international
2026

Cancer Risk in Patients with Acromegaly: Insights from a Single Center in Ankara.

Journal of clinical medicine
2026

Beyond Chordoma: A Comprehensive Review of Sacral Lesions.

Current oncology (Toronto, Ont.)
2026

CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.

Cells
2026

Comparative outcomes of anterior-posterior versus posterior-only approaches for lumbosacral chordoma: A meta-analysis of recurrence, survival, and complications.

Neurosurgical review
2026

A Clival Mass Posing a Diagnostic Challenge: Pituitary Adenoma With Rathke Cleft Cyst Mimicking Chordoma.

Cureus
2026

Clarifying methods of identifying chordoma patients from administrative billing codes.

Clinical neurology and neurosurgery
2026

Matrix Metalloproteinase-13 Is an Unfavorable Prognostic Factor in Chordoma by Digesting Growth Inhibitory Collagens.

Cancer medicine
2026

Immunotherapy and tyrosine kinase inhibitors in chordoma: a real-world data study from a European Reference Network on Rare Adult Solid Cancers member center.

Frontiers in oncology
2026

Cholesterol Metabolism Emerges as a Therapeutic Target in Chordoma.

Neuro-oncology
2026

Soft tissue chordoma of the right thigh demonstrated on 18F-FDG PET/CT and MRI: A case report.

Medicine
2026

A Dangerous Region Generation Method for Computer-Assisted Pelvic Bone Tumor Resection Surgery: A Retrospective Study.

Journal of clinical medicine
2026

A Systematic Review of Cutaneous Involvement in Metastatic Bone Sarcomas: Insights from 102 Reported Cases.

Cancers
2026

Carbon fiber-reinforced polyetheretherketone spinal instrumentation in pediatric spinal tumors: report of two cases and review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2026

Surgical Management of Large Sacral Spine Chordomas.

Indian journal of surgical oncology
2026

Caecal volvulus: A rare cause of bowel obstruction.

Current problems in surgery
2026

Hemicorporectomy: a case report on a last-resort surgical procedure.

World journal of surgical oncology
2026

Notch1 O-GlcNAcylation drives tumor stemness and mechanoadaptation to a stiff microenvironment and promotes chordoma recurrence.

The Journal of clinical investigation
2025

Stereotactic Body Radiosurgery Associated With Rectal Displacement using Gelfoam for Sacral Chordomas: Preliminary Report of 3 Cases.

Advances in radiation oncology
2026

Case Report: Anlotinib for advanced chordoma.

Frontiers in oncology
2026

Local Control With Moderately Hypofractionated Definitive Radiotherapy Delivered With a Simultaneous Integrated Boost Technique to Non-extremity Soft Tissue and Bone Sarcomas.

Clinical oncology (Royal College of Radiologists (Great Britain))
2026

Decoding the ERS-CAF immunoregulatory axis via multimodal AI and its pan-cancer prognostic and therapeutic predictive value.

NPJ digital medicine
2026

Impact of surgical margins and radiation therapy on survival in spinal chordoma and chondrosarcoma: a single-institution retrospective cohort study.

Journal of neurosurgery. Spine
2026

Cervical spine chordomas: surgical outcome assessment in a multicenter cohort from the Primary Tumor Research and Outcomes Network.

Journal of neurosurgery. Spine
2026

Pediatric chordoma - the importance of recognizing the poorly differentiated subtype: Reply to Inarejos et al.

Pediatric radiology
2026

CircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.

International journal of molecular sciences
2026

Extra-axial chordoma in the distal fibula and the importance of ankle reconstruction.

World journal of surgical oncology
2026

Electrical stimulation properties of carbon fiber versus titanium pedicle screw instrumentation: illustrative case.

Journal of neurosurgery. Case lessons
2026

Integrated immune profiling of chordomas reveals spatially organized niches and functional heterogeneity.

Neuro-oncology
2026

Nonoperative management of sacral chordomas: A systematic review of the literature.

Journal of orthopaedics
2026

Recent Advances in Proton Therapy for the Treatment of Skull Base Chordoma.

Recent patents on anti-cancer drug discovery
2026

A Needle in a Haystack: The Precision of Liquid Biopsy in Finding Bone Tumors.

Anti-cancer agents in medicinal chemistry
2025

Mucinous adenocarcinoma transformed from tailgut cyst: a case report and review of the literature.

Frontiers in oncology
2026

Thoracic chordoma following intracranial meningioma in a patient with a novel germline SMARCE1 variant.

European journal of medical genetics
2026

Advances in Clinical Trials of Boron Neutron Capture Therapy.

Research (Washington, D.C.)
2026

Insights From the AO Spine Knowledge Forum Tumor Registries: Advancing the Understanding and Management of Primary Spine Tumors Through International Multicentric Collaboration. A Narrative Review.

Global spine journal
2026

Comparative analysis of surgical outcomes and prognostic factors between primary and recurrent sacral chordomas.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2026

Recurrent clival chordoma with spinal and pulmonary metastasis: a case report.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Microstructural modelling based on diffusion weighted imaging to guide dose painting in carbon ions for large sacral chordomas.

Physics and imaging in radiation oncology
2026

Microstructural profiling and habitat analysis to characterize treatment outcome patterns in Sacral Chordomas treated with carbon ions.

Medical physics
2025

Case Report: A rare case of bone destructive sacrococcygeal chordoma presenting as anal distension.

Frontiers in oncology
2026

Targeting the tumor immune microenvironment in chordoma: From mechanistic insights to therapeutic breakthroughs.

Biochimica et biophysica acta. Reviews on cancer
2025

Metastatic sacral chordoma presenting as a cecal polyp: a case report.

Journal of surgical case reports
2025

Evaluation of crizotinib as radiosensitizer in sacral chordoma cells: effects of combined carbon ion particle therapy.

Medical oncology (Northwood, London, England)
2025

TP53 mutations as drivers of chordoma progression and hallmarks of aggressive chordoma.

Acta neuropathologica communications
2025

AI-assisted identification of innovative phytochemicals from Aizoon canariense aimed at brachyury protein in chordoma: a computational strategy.

Scientific reports
2025

Adenocarcinoma with Lymph Node Metastases of a Recurrent Tailgut Cyst: A Report of a Rare Case.

Indian journal of surgical oncology
2025

Precision in action: Using patient-specific cutting guides for en bloc resection of large sacral chordomas - A case report.

Surgical neurology international
2025

Recurrent Nasopharyngeal Chordoma Stabilized With Afatinib: A Case Report.

Cureus
2025

Management of massive sacral chordoma: a rare and complex surgical challenge.

BMJ case reports
2025

Cholesterol-metabolic TAMs regulates tumor budding like cell subpopulation to promote chordoma stemness via BACH1/ANGPTL4/SDC4 axis.

Neuro-oncology
2025

Human eosinophils exert antitumorigenic effects on chordoma.

Chinese neurosurgical journal
2026

Role of extracellular matrix in chordoma progression and therapeutic resistance.

Critical reviews in oncology/hematology
2025

Atypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years.

Cancers
2025

Malignant extrarenal rhabdoid tumor: a case report.

Journal of medical case reports
2025

Soft tissue reconstruction after total resection of sacral chordoma: Case report and literature review-is flap repair necessary?

Current problems in surgery
2026

Bone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.

Clinical neurology and neurosurgery
2025

Inhibition of CDKs Enhances Efficacy of Anti-EGFR Therapy in Chordoma.

Molecular cancer therapeutics
2025

Endoscopic endonasal approach vs microsurgical transcranial approach in intracranial chordoma surgery: a systematic review and meta-analysis.

Neurosurgical review
2026

Gross Total Resection of the Entire Cervical Spine Tumor: A Technical Note on Surgical Planning and Design of a Novel 3D-Printed Vertebral Prosthesis for Reconstruction from Skull Base to Thoracic Vertebra.

World neurosurgery
2026

Predictors of prolonged length of hospital stay and 30-Day readmission following surgical resection of spinal Chordomas: A retrospective national cancer database analysis.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

ATF4-mediated stress response as a therapeutic vulnerability in chordoma.

Molecular oncology
2025

[Combination of posterior median and transoral approaches in surgical treatment of giant chordoma of the craniovertebral junction].

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko
2025

Quality of Life and Clinical Outcomes in Patients with Skull Base Chordoma and Chondrosarcoma Treated with Pencil-Beam Scanning Proton Therapy.

Cancers
2025

CD3L1 expression and its association with the tumor microenvironment in 62 cases of osteosarcoma and Chordoma.

Scientific reports
2026

Quantitative Susceptibility Mapping in Skull Base Chordoma: In Silico Analysis and In Vivo Application Towards Indirect Hypoxia Assessment.

Magnetic resonance in medicine
2025

Application of a rapid and accurate safe-margin volume generation method in computer-assisted bone tumor resection surgery.

Journal of orthopaedic surgery and research
2026

Pediatric Head and Neck Chordomas: A National Analysis of Prognostic Indicators and Survival Outcomes.

Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
2025

Circumferential Surgical Management of a Cervical Chordoma: A Case Report and Review of the Literature.

Cureus
2025

Unexpected Long-Term Disease Control in Lung Cancer With Sacral Metastases After Conventional Radiotherapy and Chemotherapy Despite Discontinuation of Maintenance Therapy: A Case Report.

Clinical case reports
2026

Central Skull Base Neoplasms.

Radiologic clinics of North America
2025

Comparative Outcomes of Brachyury Vaccine vs. Imatinib in Advanced Chordoma: A Mayo Clinic Experience.

Cancers
2026

Proton beam therapy for chordoma: A systematic review and meta-analysis.

Critical reviews in oncology/hematology
2026

Textural analysis as an instrument to detect differences on sacral chordoma MRI post-radiation therapy.

Skeletal radiology
2025

A New Area of Neurosurgery: First Robotic Neurosurgery Clinical Case Series.

Operative neurosurgery (Hagerstown, Md.)
2025

Stereotactic radiosurgery (SRS) for primary spinal bone tumors: a systematic review and meta-analysis.

Neurosurgical review
2025

Systematic review of novel target therapies and clinical trials in chordoma.

Clinical neurology and neurosurgery
2025

Dynamic Contrast Enhanced MR Perfusion and Diffusion-Weighted Imaging of Marrow-Replacing Disorders of the Spine: A Comprehensive Review.

Neurosurgery clinics of North America
2025

Posterior Mediastinal Chordoma Resected With Anterior Thoracic Vertebral Body Through Prone Bilateral Thoracic Approaches.

Annals of thoracic surgery short reports
2025

Real-time prognosis prediction with conditional survival analysis for skull base chordoma based on SEER.

Translational cancer research
2025

Cervical chordoma en bloc resection and staged suprascapular nerve transfer reanimation: illustrative case.

Journal of neurosurgery. Case lessons
2025

Effectiveness of Preoperative Antiseptic Preparation in Transnasal Skull Base Surgery: A Randomized Three-Arm Controlled Trial.

Journal of neurological surgery. Part B, Skull base
2025

The Immune Microenvironment in Chordoma: Implications for Future Treatment.

World neurosurgery
2025

Navigation-guided resection of sacrococcygeal tumors: Case series with clinical and functional outcomes.

Medicine
2025

Ubiquitin-independent pathway regulates the RIT1-MAPK pathway in chordoma progression.

Cell death &amp; disease
2025

[Surgical techniques for the safe and rapid resection of primary or secondary sacral tumors located between the second and fourth sacral vertebrae].

Zhonghua zhong liu za zhi [Chinese journal of oncology]
2025

A refined treatment strategy for skull base chordoma: A protocol and management algorithm.

Neurosurgical review
2025

Case Report: Cranial intradural chordoma following extradural spinal chordoma.

Frontiers in surgery
2025

Random survival forests-based survival prediction for spinal chordomas.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Endoscopic endonasal transsphenoidal surgery in children: widening the spectrum of oncological indications in the pediatric age group.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Endoscopic Endonasal Supratotal Resection of a Pediatric Clival Chordoma With Intradural Extension and Brainstem Compression.

Operative neurosurgery (Hagerstown, Md.)
2025

Endoscopic Endonasal Transnasopharyngeal Approach for Ventral Craniovertebral Junction Lesions: A Technical Note.

Neurospine
2025

Machine learning-enhanced normal tissue complication probability modeling for late sciatic nerve toxicity prediction in carbon-ion radiotherapy: model development and clinical validation.

Physics in medicine and biology
2025

A clinical analysis of 19 cases of chordomas with poor differentiation treated using diverse therapeutic modalities.

Discover oncology
2025

Proton beam radiation therapy vs. photon radiation therapy and the overall survival of adult and pediatric patients diagnosed with sarcoma.

Frontiers in oncology
2026

Extra-axial chordoma of the distal femur: a clinical and radiological challenge-case report and literature review.

Skeletal radiology
2025

Analysis of long-term TNF-alpha induced EGFR tyrosine kinase inhibitor resistance in chordoma.

RSC medicinal chemistry
2025

First-in-Human Phase II Clinical Trial of Multiplex IntraTumoral Immunotherapy (MITI) in Patients with Metastatic Solid Cancer (Abscopal 5001 Trial).

Cancers
2025

Extreme Lateral Transodontoid and Extended Endonasal Transclival-Contralateral Transmaxillary Approaches for a Large Pediatric Clival-Cervical Chordoma: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2025

Late recurrence of cervical spine chordoma: illustrative case.

Journal of neurosurgery. Case lessons
2025

Collision Tumor of the Clivus: Chordoma and Chronic Lymphocytic Leukemia.

Journal of neurological surgery. Part A, Central European neurosurgery
2025

Phase I results on the efficacy, safety and pharmacokinetics of lurbinectedin and irinotecan in advanced solid tumors.

Investigational new drugs
2025

Spinal and Sacral Chordomas: Overview With Imaging Review.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
2025

Carbon Ion and Proton Therapy in Sacral Chordoma: A Systematic Review.

Journal of clinical medicine
2025

Evaluating the technical feasibility of biology-guided dose painting in proton therapy.

Physics and imaging in radiation oncology
2025

Improving oncological outcomes for pelvic bone sarcomas: Is it possible?

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
2025

The effect of TERT promoter mutation on predicting meningioma outcomes: a multi-institutional cohort analysis.

The Lancet. Oncology
2025

Selective targeting of TBXT with DARPins identifies regulatory networks and therapeutic vulnerabilities in chordoma.

Science advances
2025

Computer vision based efficient segmentation and classification of multi brain tumor using computed tomography images.

Scientific reports
2026

Loss of CD90 alters EMT-associated features and drug sensitivity in U-CH1 chordoma cells.

Naunyn-Schmiedeberg's archives of pharmacology
2025

Cytomorphological Features of Fine Needle Aspiration in 11 Conventional and 2 Dedifferentiated Chordomas: Correlation With Histopathology.

Diagnostic cytopathology
2025

Space-making Particle Therapy Using Bioabsorbable Spacer for Bone and Soft Tissue Sarcomas: Two-institution Experience.

Anticancer research
2025

Sacrococcygeal Chordoma: A Diagnostic Challenge.

Cureus
2026

Two-Stage Excision of Advanced, Infiltrative Sacral Chordoma.

The American surgeon
2025

Cervical Spine Chordoma With Tracheal and Vascular Invasion: A Case Report.

Cureus
2025

Impact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma.

Journal of clinical medicine
2025

Distinctive Characteristics of Rare Sellar Lesions Mimicking Pituitary Adenomas: A Collection of Unusual Neoplasms.

Cancers
2025

Recurrence Patterns After Resection of Sacral Chordoma: Toward an Optimized Postoperative Target Volume Definition.

Cancers
2025

Predicting sacral insufficiency fractures in carbon ion therapy for sacral chordoma: The role of RBE-weighted dose, dose-averaged LET, and volume.

Medical physics
2025

Mutational analysis of primary and advanced chordoma tissue using next-generation sequencing.

Cancer
2025

How to foster new treatment development in ultra-rare tumours? Joint EMA-EORTC multi-stakeholder workshops on ultra-rare sarcomas as a model for rare cancers.

Cancer treatment reviews
2025

Milademetan in Advanced Solid Tumors with MDM2 Amplification and Wild-type TP53: Preclinical and Phase II Clinical Trial Results.

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Pitfalls and strategies of CAR-T therapy in solid tumors and implications for chordoma treatment.

Immunotherapy
2025

Aortitis after granulocyte-colony stimulating factor administration: A case report.

Biomedical reports
2026

Ectopic clival craniopharyngioma.

Neuroradiology
2025

Acute left and right ventricular heart failure with transient myocardial edema in a 26-Year-old female patient with peripartum cardiomyopathy and clivus chordoma.

The international journal of cardiovascular imaging
2025

Occipitocervical surgery rescue: The "Catcher's Mitt" technique.

Journal of craniovertebral junction &amp; spine
2025

Poorly Differentiated Aggressive Sacral Chordoma With Upfront Lung Metastases in a Child-A Case Report.

Clinical case reports
2025

Insights Into Non-Classic Primary Bone Sarcomas in Children, Adolescents, and Young Adults: A French Descriptive Study.

Pediatric blood &amp; cancer
2025

How precise is excision and reconstruction using 3D printing technology for total sacrectomy: accuracy validation in 9 consecutive cases.

3D printing in medicine
2025

The Spinal Chordoma Patient Reported Outcome Survey (spCPROS): a patient-centered, disease-specific tool for assessing quality of life in spinal chordoma patients.

Journal of neuro-oncology
2025

Bone Lesions of the Head and Neck.

Surgical pathology clinics
2025

Resection of C2 chordoma after failed ion-beam radiotherapy: illustrative case.

Journal of neurosurgery. Case lessons
2025

Corrigendum to: Integrating single-cell and spatial transcriptomics reveals endoplasmic reticulum stress-related CAF subpopulations associated with chordoma progression.

Neuro-oncology
2025

Dedifferentiated Chordoma Harboring a Novel KRAS (G13D) Hot Spot Missense Mutation and an Additional Novel RPSAP52-HMGA2 Fusion: A Case Study.

Neuropathology : official journal of the Japanese Society of Neuropathology
2025

Long-Term Clinical Outcome and Quality of Life of Children, Adolescents, and Young Adults With Chordoma or Chondrosarcoma Treated With Pencil Beam Scanning Proton Therapy.

Pediatric blood &amp; cancer
2025

Overcoming Radiotherapy Resistance in Chordoma through the Synergistic Action of Selenium-Containing Nanoparticles and THZ1.

ACS nano
2025

[Interpretation of important issues of the secondary clinical practice guideline on management of primary malignant bone tumors by the Japanese Orthopaedic Association].

Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery
2025

Multidisciplinary En-Bloc Resection of Sacral Chordoma: A Narrative Review and Illustrative Case.

Journal of clinical medicine
2025

Long-term functional outcomes and their relationship to nerve root sacrifice after definitive surgery for sacrococcygeal chordoma.

Journal of neurosurgery. Spine
2025

Phase Ib Study of Pembrolizumab in Combination with Intratumoral Injection of Clostridium novyi-NT in Patients with Advanced Solid Tumors.

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Lower Cervical Chordomas: A Case Report and Differential Diagnosis.

Cancer reports (Hoboken, N.J.)
2025

CDK4/6 inhibition in advanced chordoma: final results of the NCT PMO-1601 trial.

ESMO open
2025

Extra Axial Chordoma of the Distal Tibia: A Case Report.

JBJS case connector
2025

Endoscopic Endonasal Resection of Clival Chordomas.

Advances and technical standards in neurosurgery
2025

Tumor control probability in large sacral chordomas treated with carbon ions radiotherapy integrating advanced microstructural modelling.

Physica medica : PM : an international journal devoted to the applications of physics to medicine and biology : official journal of the Italian Association of Biomedical Physics (AIFB)
2025

Pansarcoma Analysis of Cyclin-Dependent Kinase and Cyclin Outlier Gene Expression Highlights CDK7 as a Potential Therapeutic Target in Chordoma.

JCO precision oncology
2025

Case Report: Collision tumors of the sellar region: pituitary adenoma and chordoma coexist in the sellar region.

Frontiers in endocrinology
2025

Hemicorporectomy in the ICU: a complex case report.

BMC anesthesiology
2025

Cold atmospheric plasma effectively kills chordoma cells through induction of intracellular reactive oxygen species.

Scientific reports
2026

Safety Profile of Select Perioperative Lumbar Drain Use After Endoscopic Endonasal Surgery.

Operative neurosurgery (Hagerstown, Md.)
2025

Chordoma: Site-Wise Predilections and Pathological Tenets.

Journal of the West African College of Surgeons
2025

Long-term Efficacy and Safety of Carbon-ion Radiotherapy for Thoracic Chordoma: A Case Report.

In vivo (Athens, Greece)
2025

EEA for sellar chodrosarcomas: case series with literature review.

Chinese neurosurgical journal
2025

High-intensity focused ultrasound treatment of unresectable soft tissue sarcoma and desmoid tumours - a systematic review.

Clinical radiology
2025

Functional Outcomes and Activity Levels in Patients After Internal Hemipelvectomy for Primary Sarcoma Involving the Bony Pelvis.

Diagnostics (Basel, Switzerland)
2025

As Radical as Technically Feasible-Surgical Treatment for Mobile Spine Chordoma.

Cancers
2025

Impact of proton versus photon radiotherapy on overall survival in the management of spinal chordoma and mortality risk prediction: a nationwide analysis.

Journal of neuro-oncology
2025

Outcomes after total en bloc spondylectomy at a mean follow-up of 11 years.

Journal of neurosurgery. Spine
2026

The gentle giant - A case sacrococcygeal chordoma.

RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin
2026

Machine learning-based models for outcome prediction in skull base and spinal chordomas: a systematic review and meta-analysis.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Identification of immune subtypes associated with the prognosis in skull base chordoma.

Acta neuropathologica communications
2026

Characterization of Soft Tissue Reconstruction Following Chordoma Resection.

Journal of reconstructive microsurgery
2025

Robotic Radiosurgery for Sacral Chordoma: Preserving Function in a Clinical Conundrum Scenario.

Cureus
2025

SMARCB1 orchestrates cellular plasticity and oncogenic pathways in typical and chondroid chordomas.

Scientific reports
2025

Risk factors for mechanical complications following spondylectomy of thoracolumbar primary spinal column tumors.

Journal of neurosurgery. Spine
2025

Artificial intelligence and chordoma: A scoping review of the current landscape and future directions.

Brain &amp; spine
2025

Layered Repair in Extensive Sacral Defects with Bilaminar Gluteal Flaps: Demirdover Flaps.

Plastic and reconstructive surgery. Global open
2025

A "Don't Touch" Lesion: Arrested Pneumatization of the Sphenoid Mimicking Chordoma.

Ear, nose, &amp; throat journal
2025

Notochord-derived lesion presenting with cerebrospinal fluid rhinorrhea: illustrative case.

Journal of neurosurgery. Case lessons
2025

Radiogenomics and radiomics of skull base chordoma: Classification of novel radiomic subgroups and prediction of genetic signatures and clinical outcomes.

Neuro-oncology
2025

Risk analysis for delayed cerebrospinal fluid leak as a late complication of endoscopic transnasal surgery: effects of irradiation and insights into reconstruction methods.

Neurosurgical review
2025

Clinical Differences Among Histological Categories of Sarcoma: Insights from 97,062 Patients.

Cancers
2025

Bilateral anteromedial petrosectomy via endoscopic endonasal transclival approach for calcified sphenopetroclival chondrosarcoma with petrous apices and cavernous sinus involvement: technical nuances of a minimally invasive solution. Illustrative case.

Journal of neurosurgery. Case lessons
2025

Exploring perspectives on skull base chordoma management: a modified Delphi approach to consensus.

Journal of neuro-oncology
2025

Staged approach to managing aggressive clival lesions extending into occipital condyles illustrated by a rare urothelial carcinoma metastasis: illustrative case.

Journal of neurosurgery. Case lessons
2025

Cracking Chordoma's Conundrum: Immune Checkpoints Provide a Potential Modality.

International journal of medical sciences
2025

Total Resection of Clival Chordoma with Brainstem Invasion via Endoscopic Transnasal Approach: Technical Note.

World neurosurgery
2025

First-in-human study of FAZ053, an anti-programmed death-ligand 1 (anti-PD-L1) monoclonal antibody, alone and in combination with spartalizumab (anti-PD-1), in patients with advanced malignancies.

ESMO open
2025

Skull base chordomas - emphasis on surgical strategy and recurrence-free survival.

Medicine and pharmacy reports
2026

Combined 6th and 12th Cranial Nerve Palsies (Godtfredsen Syndrome) due to Clival Lesions.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2025

Cost-Effectiveness of Carbon Ion Radiotherapy in Oncology: A Systematic Review.

Cureus
2025

Pelvic and Perineal Reconstruction After Bowel, Gynecological or Sacral Tumor Resection: A Case Series.

Journal of clinical medicine
2025

TFCP2 Fusion-Positive Rhabdomyosarcomas: A Report of 10 Cases and a Review of the Literature.

Cancers
2026

Low FDG Uptake of a Mass Initially Suspected of Being a Nasopharyngeal Carcinoma: MRI and FDG PET/CT Reveal a Clivus Chordoma.

Clinical nuclear medicine
Ver todos os 2.904 no EuropePMC

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Transdural Cisternotomy in the Extended Middle Fossa Approach.
    Neurology India· 2026· PMID 41817072mais citado
  2. Surgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.
    Surgical neurology international· 2026· PMID 41783173mais citado
  3. CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.
    Cells· 2026· PMID 41744820mais citado
  4. CircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.
    International journal of molecular sciences· 2026· PMID 41596634mais citado
  5. Bone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.
    Clinical neurology and neurosurgery· 2026· PMID 41349423mais citado
  6. CT perfusion-focused preoperative assessment of the blood-supplying state among imaging modalities in skull base chordoma: a retrospective single-center cohort.
    J Neurooncol· 2026· PMID 41973299recente
  7. Sacral chordomas: surgical management, reconstruction, and translational advances: a systematic review.
    J Spine Surg· 2026· PMID 41971904recente
  8. Immune and stromal signaling networks in chordoma: an evidence-weighted review of ligand-receptor interactions and therapeutic implications.
    Syst Rev· 2026· PMID 41965872recente
  9. Skull-base chordoma in a dog.
    J Vet Diagn Invest· 2026· PMID 41954236recente
  10. Extra-axial chordoma of the distal tibia incidentally discovered during fixation of a presumed pathologic fracture.
    J Surg Case Rep· 2026· PMID 41939238recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:178(Orphanet)
  2. OMIM OMIM:215400(OMIM)
  3. MONDO:0008978(MONDO)
  4. GARD:1303(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Artigo Wikipedia(Wikipedia)
  8. Q1076389(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Cordoma
Compêndio · Raras BR

Cordoma

ORPHA:178 · MONDO:0008978
Prevalência
<1 / 1 000 000
Herança
Autosomal dominant, Not applicable
CID-10
C76.7 · Neoplasia maligna de outras localizações mal definidas
CID-11
Ensaios
23 ativos
Medicamentos
10 registrados
Início
Adult
Prevalência
0.06 (United States)
MedGen
UMLS
C0008487
EuropePMC
Wikidata
Wikipedia
Papers 10a
Evidência
🥇 Rev. sistemática
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