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.
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.
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Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 7 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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.
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
Nucleus
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.
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
Nucleus
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.
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
NucleusCytoplasm, cytoskeleton, microtubule organizing center, centrosome
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.
Medicamentos e terapias
Mecanismo: Receptor protein-tyrosine kinase erbB-2 inhibitor
Mecanismo: CDK6/cyclin D1 inhibitor
Mecanismo: Programmed cell death protein 1 inhibitor
Mecanismo: Tyrosine-protein kinase ABL inhibitor
Mecanismo: Epidermal growth factor receptor erbB1 inhibitor
Mecanismo: Lymphocyte activation gene 3 protein inhibitor
Mecanismo: GAR transformylase inhibitor
Mecanismo: Tyrosine-protein kinase ABL inhibitor
Mecanismo: Programmed cell death 1 ligand 1 other
Mecanismo: Granulocyte colony stimulating factor receptor agonist
Variantes genéticas (ClinVar)
24,454 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 17 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
22 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Cordoma
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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
Ensaios em destaque
🟢 Recrutando agora
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Outros ensaios clínicos
75 ensaios clínicos encontrados, 23 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 2.178
Transdural Cisternotomy in the Extended Middle Fossa Approach.
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.
Surgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.
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.
CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.
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.
CircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.
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.
Bone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.
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
CT perfusion-focused preoperative assessment of the blood-supplying state among imaging modalities in skull base chordoma: a retrospective single-center cohort.
Sacral chordomas: surgical management, reconstruction, and translational advances: a systematic review.
🥉 Relato de casoImmune and stromal signaling networks in chordoma: an evidence-weighted review of ligand-receptor interactions and therapeutic implications.
Skull-base chordoma in a dog.
🥇 Revisão sistemáticaExtra-axial chordoma of the distal tibia incidentally discovered during fixation of a presumed pathologic fracture.
📚 EuropePMC2.904 artigos no totalmostrando 197
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 SurgeryChopsticks mononostril approach with sphenoid sinus cranialization and septal mucosa suturing: a new minimally invasive concept and closure technique for endoscopic endonasal approaches.
Journal of neurosurgeryOutcomes of the Translabyrinthine-Infralabyrinthine Approach for Resection of Jugular Foramen Chordomas.
The Journal of craniofacial surgeryHearing Outcomes in the Surgical Management of Petroclival Malignancies: Understanding the Impact of Surgical Strategy and Adjuvant Radiotherapy.
Operative neurosurgery (Hagerstown, Md.)Advances in Radiation Therapy for Primary Bone Malignancies.
Technology in cancer research & treatmentTransdural Cisternotomy in the Extended Middle Fossa Approach.
Neurology IndiaIntraosseous Pseudoprogression vs. Progression and Recurrence of Clival Chordoma after Proton Radiation Therapy: Case Series.
Journal of neurological surgery. Part B, Skull baseIndividual and Community-Level Social Determinant Associations with Skull Base Chordoma and Chondrosarcoma Disparities in the United States.
Journal of neurological surgery. Part B, Skull baseAn insight into the pharmacoeconomics of carbon ion radiotherapy: a systematic review.
Radiation oncology journalParaspinal Skip Lesion Encountered During the Resection of a Sacral Chordoma: A Case Report.
CureusOncolytic Adenoviral Infection of Chordoma Achieves Treatment Efficacy Associated with Immunologic Response and Tumor Microenvironmental Alteration.
Neuro-oncologyContemporary Management of Standard Chordoma Arising in the Mobile Spine and Sacrum.
The Journal of the American Academy of Orthopaedic SurgeonsSurgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.
Surgical neurology internationalCancer Risk in Patients with Acromegaly: Insights from a Single Center in Ankara.
Journal of clinical medicineBeyond Chordoma: A Comprehensive Review of Sacral Lesions.
Current oncology (Toronto, Ont.)CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.
CellsComparative outcomes of anterior-posterior versus posterior-only approaches for lumbosacral chordoma: A meta-analysis of recurrence, survival, and complications.
Neurosurgical reviewA Clival Mass Posing a Diagnostic Challenge: Pituitary Adenoma With Rathke Cleft Cyst Mimicking Chordoma.
CureusClarifying methods of identifying chordoma patients from administrative billing codes.
Clinical neurology and neurosurgeryMatrix Metalloproteinase-13 Is an Unfavorable Prognostic Factor in Chordoma by Digesting Growth Inhibitory Collagens.
Cancer medicineImmunotherapy 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 oncologyCholesterol Metabolism Emerges as a Therapeutic Target in Chordoma.
Neuro-oncologySoft tissue chordoma of the right thigh demonstrated on 18F-FDG PET/CT and MRI: A case report.
MedicineA Dangerous Region Generation Method for Computer-Assisted Pelvic Bone Tumor Resection Surgery: A Retrospective Study.
Journal of clinical medicineA Systematic Review of Cutaneous Involvement in Metastatic Bone Sarcomas: Insights from 102 Reported Cases.
CancersCarbon 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 NeurosurgerySurgical Management of Large Sacral Spine Chordomas.
Indian journal of surgical oncologyCaecal volvulus: A rare cause of bowel obstruction.
Current problems in surgeryHemicorporectomy: a case report on a last-resort surgical procedure.
World journal of surgical oncologyNotch1 O-GlcNAcylation drives tumor stemness and mechanoadaptation to a stiff microenvironment and promotes chordoma recurrence.
The Journal of clinical investigationStereotactic Body Radiosurgery Associated With Rectal Displacement using Gelfoam for Sacral Chordomas: Preliminary Report of 3 Cases.
Advances in radiation oncologyCase Report: Anlotinib for advanced chordoma.
Frontiers in oncologyLocal 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))Decoding the ERS-CAF immunoregulatory axis via multimodal AI and its pan-cancer prognostic and therapeutic predictive value.
NPJ digital medicineImpact of surgical margins and radiation therapy on survival in spinal chordoma and chondrosarcoma: a single-institution retrospective cohort study.
Journal of neurosurgery. SpineCervical spine chordomas: surgical outcome assessment in a multicenter cohort from the Primary Tumor Research and Outcomes Network.
Journal of neurosurgery. SpinePediatric chordoma - the importance of recognizing the poorly differentiated subtype: Reply to Inarejos et al.
Pediatric radiologyCircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.
International journal of molecular sciencesExtra-axial chordoma in the distal fibula and the importance of ankle reconstruction.
World journal of surgical oncologyElectrical stimulation properties of carbon fiber versus titanium pedicle screw instrumentation: illustrative case.
Journal of neurosurgery. Case lessonsIntegrated immune profiling of chordomas reveals spatially organized niches and functional heterogeneity.
Neuro-oncologyNonoperative management of sacral chordomas: A systematic review of the literature.
Journal of orthopaedicsRecent Advances in Proton Therapy for the Treatment of Skull Base Chordoma.
Recent patents on anti-cancer drug discoveryA Needle in a Haystack: The Precision of Liquid Biopsy in Finding Bone Tumors.
Anti-cancer agents in medicinal chemistryMucinous adenocarcinoma transformed from tailgut cyst: a case report and review of the literature.
Frontiers in oncologyThoracic chordoma following intracranial meningioma in a patient with a novel germline SMARCE1 variant.
European journal of medical geneticsAdvances in Clinical Trials of Boron Neutron Capture Therapy.
Research (Washington, D.C.)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 journalComparative 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 SocietyRecurrent 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 SocietyMicrostructural modelling based on diffusion weighted imaging to guide dose painting in carbon ions for large sacral chordomas.
Physics and imaging in radiation oncologyMicrostructural profiling and habitat analysis to characterize treatment outcome patterns in Sacral Chordomas treated with carbon ions.
Medical physicsCase Report: A rare case of bone destructive sacrococcygeal chordoma presenting as anal distension.
Frontiers in oncologyTargeting the tumor immune microenvironment in chordoma: From mechanistic insights to therapeutic breakthroughs.
Biochimica et biophysica acta. Reviews on cancerMetastatic sacral chordoma presenting as a cecal polyp: a case report.
Journal of surgical case reportsEvaluation of crizotinib as radiosensitizer in sacral chordoma cells: effects of combined carbon ion particle therapy.
Medical oncology (Northwood, London, England)TP53 mutations as drivers of chordoma progression and hallmarks of aggressive chordoma.
Acta neuropathologica communicationsAI-assisted identification of innovative phytochemicals from Aizoon canariense aimed at brachyury protein in chordoma: a computational strategy.
Scientific reportsAdenocarcinoma with Lymph Node Metastases of a Recurrent Tailgut Cyst: A Report of a Rare Case.
Indian journal of surgical oncologyPrecision in action: Using patient-specific cutting guides for en bloc resection of large sacral chordomas - A case report.
Surgical neurology internationalRecurrent Nasopharyngeal Chordoma Stabilized With Afatinib: A Case Report.
CureusManagement of massive sacral chordoma: a rare and complex surgical challenge.
BMJ case reportsCholesterol-metabolic TAMs regulates tumor budding like cell subpopulation to promote chordoma stemness via BACH1/ANGPTL4/SDC4 axis.
Neuro-oncologyHuman eosinophils exert antitumorigenic effects on chordoma.
Chinese neurosurgical journalRole of extracellular matrix in chordoma progression and therapeutic resistance.
Critical reviews in oncology/hematologyAtypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years.
CancersMalignant extrarenal rhabdoid tumor: a case report.
Journal of medical case reportsSoft tissue reconstruction after total resection of sacral chordoma: Case report and literature review-is flap repair necessary?
Current problems in surgeryBone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.
Clinical neurology and neurosurgeryInhibition of CDKs Enhances Efficacy of Anti-EGFR Therapy in Chordoma.
Molecular cancer therapeuticsEndoscopic endonasal approach vs microsurgical transcranial approach in intracranial chordoma surgery: a systematic review and meta-analysis.
Neurosurgical reviewGross 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 neurosurgeryPredictors 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 AustralasiaATF4-mediated stress response as a therapeutic vulnerability in chordoma.
Molecular oncology[Combination of posterior median and transoral approaches in surgical treatment of giant chordoma of the craniovertebral junction].
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoQuality of Life and Clinical Outcomes in Patients with Skull Base Chordoma and Chondrosarcoma Treated with Pencil-Beam Scanning Proton Therapy.
CancersCD3L1 expression and its association with the tumor microenvironment in 62 cases of osteosarcoma and Chordoma.
Scientific reportsQuantitative Susceptibility Mapping in Skull Base Chordoma: In Silico Analysis and In Vivo Application Towards Indirect Hypoxia Assessment.
Magnetic resonance in medicineApplication of a rapid and accurate safe-margin volume generation method in computer-assisted bone tumor resection surgery.
Journal of orthopaedic surgery and researchPediatric 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 SurgeryCircumferential Surgical Management of a Cervical Chordoma: A Case Report and Review of the Literature.
CureusUnexpected 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 reportsCentral Skull Base Neoplasms.
Radiologic clinics of North AmericaComparative Outcomes of Brachyury Vaccine vs. Imatinib in Advanced Chordoma: A Mayo Clinic Experience.
CancersProton beam therapy for chordoma: A systematic review and meta-analysis.
Critical reviews in oncology/hematologyTextural analysis as an instrument to detect differences on sacral chordoma MRI post-radiation therapy.
Skeletal radiologyA New Area of Neurosurgery: First Robotic Neurosurgery Clinical Case Series.
Operative neurosurgery (Hagerstown, Md.)Stereotactic radiosurgery (SRS) for primary spinal bone tumors: a systematic review and meta-analysis.
Neurosurgical reviewSystematic review of novel target therapies and clinical trials in chordoma.
Clinical neurology and neurosurgeryDynamic Contrast Enhanced MR Perfusion and Diffusion-Weighted Imaging of Marrow-Replacing Disorders of the Spine: A Comprehensive Review.
Neurosurgery clinics of North AmericaPosterior Mediastinal Chordoma Resected With Anterior Thoracic Vertebral Body Through Prone Bilateral Thoracic Approaches.
Annals of thoracic surgery short reportsReal-time prognosis prediction with conditional survival analysis for skull base chordoma based on SEER.
Translational cancer researchCervical chordoma en bloc resection and staged suprascapular nerve transfer reanimation: illustrative case.
Journal of neurosurgery. Case lessonsEffectiveness of Preoperative Antiseptic Preparation in Transnasal Skull Base Surgery: A Randomized Three-Arm Controlled Trial.
Journal of neurological surgery. Part B, Skull baseThe Immune Microenvironment in Chordoma: Implications for Future Treatment.
World neurosurgeryNavigation-guided resection of sacrococcygeal tumors: Case series with clinical and functional outcomes.
MedicineUbiquitin-independent pathway regulates the RIT1-MAPK pathway in chordoma progression.
Cell death & disease[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]A refined treatment strategy for skull base chordoma: A protocol and management algorithm.
Neurosurgical reviewCase Report: Cranial intradural chordoma following extradural spinal chordoma.
Frontiers in surgeryRandom survival forests-based survival prediction for spinal chordomas.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaEndoscopic 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 NeurosurgeryEndoscopic Endonasal Supratotal Resection of a Pediatric Clival Chordoma With Intradural Extension and Brainstem Compression.
Operative neurosurgery (Hagerstown, Md.)Endoscopic Endonasal Transnasopharyngeal Approach for Ventral Craniovertebral Junction Lesions: A Technical Note.
NeurospineMachine 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 biologyA clinical analysis of 19 cases of chordomas with poor differentiation treated using diverse therapeutic modalities.
Discover oncologyProton beam radiation therapy vs. photon radiation therapy and the overall survival of adult and pediatric patients diagnosed with sarcoma.
Frontiers in oncologyExtra-axial chordoma of the distal femur: a clinical and radiological challenge-case report and literature review.
Skeletal radiologyAnalysis of long-term TNF-alpha induced EGFR tyrosine kinase inhibitor resistance in chordoma.
RSC medicinal chemistryFirst-in-Human Phase II Clinical Trial of Multiplex IntraTumoral Immunotherapy (MITI) in Patients with Metastatic Solid Cancer (Abscopal 5001 Trial).
CancersExtreme Lateral Transodontoid and Extended Endonasal Transclival-Contralateral Transmaxillary Approaches for a Large Pediatric Clival-Cervical Chordoma: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)Late recurrence of cervical spine chordoma: illustrative case.
Journal of neurosurgery. Case lessonsCollision Tumor of the Clivus: Chordoma and Chronic Lymphocytic Leukemia.
Journal of neurological surgery. Part A, Central European neurosurgeryPhase I results on the efficacy, safety and pharmacokinetics of lurbinectedin and irinotecan in advanced solid tumors.
Investigational new drugsSpinal and Sacral Chordomas: Overview With Imaging Review.
Journal of neuroimaging : official journal of the American Society of NeuroimagingCarbon Ion and Proton Therapy in Sacral Chordoma: A Systematic Review.
Journal of clinical medicineEvaluating the technical feasibility of biology-guided dose painting in proton therapy.
Physics and imaging in radiation oncologyImproving 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 OncologyThe effect of TERT promoter mutation on predicting meningioma outcomes: a multi-institutional cohort analysis.
The Lancet. OncologySelective targeting of TBXT with DARPins identifies regulatory networks and therapeutic vulnerabilities in chordoma.
Science advancesComputer vision based efficient segmentation and classification of multi brain tumor using computed tomography images.
Scientific reportsLoss of CD90 alters EMT-associated features and drug sensitivity in U-CH1 chordoma cells.
Naunyn-Schmiedeberg's archives of pharmacologyCytomorphological Features of Fine Needle Aspiration in 11 Conventional and 2 Dedifferentiated Chordomas: Correlation With Histopathology.
Diagnostic cytopathologySpace-making Particle Therapy Using Bioabsorbable Spacer for Bone and Soft Tissue Sarcomas: Two-institution Experience.
Anticancer researchSacrococcygeal Chordoma: A Diagnostic Challenge.
CureusTwo-Stage Excision of Advanced, Infiltrative Sacral Chordoma.
The American surgeonCervical Spine Chordoma With Tracheal and Vascular Invasion: A Case Report.
CureusImpact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma.
Journal of clinical medicineDistinctive Characteristics of Rare Sellar Lesions Mimicking Pituitary Adenomas: A Collection of Unusual Neoplasms.
CancersRecurrence Patterns After Resection of Sacral Chordoma: Toward an Optimized Postoperative Target Volume Definition.
CancersPredicting sacral insufficiency fractures in carbon ion therapy for sacral chordoma: The role of RBE-weighted dose, dose-averaged LET, and volume.
Medical physicsMutational analysis of primary and advanced chordoma tissue using next-generation sequencing.
CancerHow 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 reviewsMilademetan 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 ResearchPitfalls and strategies of CAR-T therapy in solid tumors and implications for chordoma treatment.
ImmunotherapyAortitis after granulocyte-colony stimulating factor administration: A case report.
Biomedical reportsEctopic clival craniopharyngioma.
NeuroradiologyAcute 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 imagingOccipitocervical surgery rescue: The "Catcher's Mitt" technique.
Journal of craniovertebral junction & spinePoorly Differentiated Aggressive Sacral Chordoma With Upfront Lung Metastases in a Child-A Case Report.
Clinical case reportsInsights Into Non-Classic Primary Bone Sarcomas in Children, Adolescents, and Young Adults: A French Descriptive Study.
Pediatric blood & cancerHow precise is excision and reconstruction using 3D printing technology for total sacrectomy: accuracy validation in 9 consecutive cases.
3D printing in medicineThe 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-oncologyBone Lesions of the Head and Neck.
Surgical pathology clinicsResection of C2 chordoma after failed ion-beam radiotherapy: illustrative case.
Journal of neurosurgery. Case lessonsCorrigendum to: Integrating single-cell and spatial transcriptomics reveals endoplasmic reticulum stress-related CAF subpopulations associated with chordoma progression.
Neuro-oncologyDedifferentiated 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 NeuropathologyLong-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 & cancerOvercoming Radiotherapy Resistance in Chordoma through the Synergistic Action of Selenium-Containing Nanoparticles and THZ1.
ACS nano[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 surgeryMultidisciplinary En-Bloc Resection of Sacral Chordoma: A Narrative Review and Illustrative Case.
Journal of clinical medicineLong-term functional outcomes and their relationship to nerve root sacrifice after definitive surgery for sacrococcygeal chordoma.
Journal of neurosurgery. SpinePhase 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 ResearchLower Cervical Chordomas: A Case Report and Differential Diagnosis.
Cancer reports (Hoboken, N.J.)CDK4/6 inhibition in advanced chordoma: final results of the NCT PMO-1601 trial.
ESMO openExtra Axial Chordoma of the Distal Tibia: A Case Report.
JBJS case connectorEndoscopic Endonasal Resection of Clival Chordomas.
Advances and technical standards in neurosurgeryTumor 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)Pansarcoma Analysis of Cyclin-Dependent Kinase and Cyclin Outlier Gene Expression Highlights CDK7 as a Potential Therapeutic Target in Chordoma.
JCO precision oncologyCase Report: Collision tumors of the sellar region: pituitary adenoma and chordoma coexist in the sellar region.
Frontiers in endocrinologyHemicorporectomy in the ICU: a complex case report.
BMC anesthesiologyCold atmospheric plasma effectively kills chordoma cells through induction of intracellular reactive oxygen species.
Scientific reportsSafety Profile of Select Perioperative Lumbar Drain Use After Endoscopic Endonasal Surgery.
Operative neurosurgery (Hagerstown, Md.)Chordoma: Site-Wise Predilections and Pathological Tenets.
Journal of the West African College of SurgeonsLong-term Efficacy and Safety of Carbon-ion Radiotherapy for Thoracic Chordoma: A Case Report.
In vivo (Athens, Greece)EEA for sellar chodrosarcomas: case series with literature review.
Chinese neurosurgical journalHigh-intensity focused ultrasound treatment of unresectable soft tissue sarcoma and desmoid tumours - a systematic review.
Clinical radiologyFunctional Outcomes and Activity Levels in Patients After Internal Hemipelvectomy for Primary Sarcoma Involving the Bony Pelvis.
Diagnostics (Basel, Switzerland)As Radical as Technically Feasible-Surgical Treatment for Mobile Spine Chordoma.
CancersImpact of proton versus photon radiotherapy on overall survival in the management of spinal chordoma and mortality risk prediction: a nationwide analysis.
Journal of neuro-oncologyOutcomes after total en bloc spondylectomy at a mean follow-up of 11 years.
Journal of neurosurgery. SpineThe gentle giant - A case sacrococcygeal chordoma.
RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der NuklearmedizinMachine 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 SocietyIdentification of immune subtypes associated with the prognosis in skull base chordoma.
Acta neuropathologica communicationsCharacterization of Soft Tissue Reconstruction Following Chordoma Resection.
Journal of reconstructive microsurgeryRobotic Radiosurgery for Sacral Chordoma: Preserving Function in a Clinical Conundrum Scenario.
CureusSMARCB1 orchestrates cellular plasticity and oncogenic pathways in typical and chondroid chordomas.
Scientific reportsRisk factors for mechanical complications following spondylectomy of thoracolumbar primary spinal column tumors.
Journal of neurosurgery. SpineArtificial intelligence and chordoma: A scoping review of the current landscape and future directions.
Brain & spineLayered Repair in Extensive Sacral Defects with Bilaminar Gluteal Flaps: Demirdover Flaps.
Plastic and reconstructive surgery. Global openA "Don't Touch" Lesion: Arrested Pneumatization of the Sphenoid Mimicking Chordoma.
Ear, nose, & throat journalNotochord-derived lesion presenting with cerebrospinal fluid rhinorrhea: illustrative case.
Journal of neurosurgery. Case lessonsRadiogenomics and radiomics of skull base chordoma: Classification of novel radiomic subgroups and prediction of genetic signatures and clinical outcomes.
Neuro-oncologyRisk analysis for delayed cerebrospinal fluid leak as a late complication of endoscopic transnasal surgery: effects of irradiation and insights into reconstruction methods.
Neurosurgical reviewClinical Differences Among Histological Categories of Sarcoma: Insights from 97,062 Patients.
CancersBilateral 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 lessonsExploring perspectives on skull base chordoma management: a modified Delphi approach to consensus.
Journal of neuro-oncologyStaged approach to managing aggressive clival lesions extending into occipital condyles illustrated by a rare urothelial carcinoma metastasis: illustrative case.
Journal of neurosurgery. Case lessonsCracking Chordoma's Conundrum: Immune Checkpoints Provide a Potential Modality.
International journal of medical sciencesTotal Resection of Clival Chordoma with Brainstem Invasion via Endoscopic Transnasal Approach: Technical Note.
World neurosurgeryFirst-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 openSkull base chordomas - emphasis on surgical strategy and recurrence-free survival.
Medicine and pharmacy reportsCombined 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 SocietyCost-Effectiveness of Carbon Ion Radiotherapy in Oncology: A Systematic Review.
CureusPelvic and Perineal Reconstruction After Bowel, Gynecological or Sacral Tumor Resection: A Case Series.
Journal of clinical medicineTFCP2 Fusion-Positive Rhabdomyosarcomas: A Report of 10 Cases and a Review of the Literature.
CancersLow FDG Uptake of a Mass Initially Suspected of Being a Nasopharyngeal Carcinoma: MRI and FDG PET/CT Reveal a Clivus Chordoma.
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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.
- Transdural Cisternotomy in the Extended Middle Fossa Approach.
- Surgical management of a large sacrococcygeal chordoma complicated by rectal perforation: A case report.
- CHI3L1 Expression in Chordoma: Implications for Immunotherapeutic Intervention.
- CircRNAs in Immuno-Metabolic Reprogramming of Chordoma Cancer: Molecular Crosstalk and Therapeutic Potential.
- Bone modifying agents and multikinase inhibitors as treatments for chordoma: A TriNetX-based retrospective cohort study.
- CT perfusion-focused preoperative assessment of the blood-supplying state among imaging modalities in skull base chordoma: a retrospective single-center cohort.
- Sacral chordomas: surgical management, reconstruction, and translational advances: a systematic review.
- Immune and stromal signaling networks in chordoma: an evidence-weighted review of ligand-receptor interactions and therapeutic implications.
- Skull-base chordoma in a dog.
- Extra-axial chordoma of the distal tibia incidentally discovered during fixation of a presumed pathologic fracture.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:178(Orphanet)
- OMIM OMIM:215400(OMIM)
- MONDO:0008978(MONDO)
- GARD:1303(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- 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
