SEARCH · PubMed Health

Results for “Chordoma”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources

PubMed1978

[Chordomas. Excision of a lumbar chordoma: 2 1/2 years survival without signs of recurrence and without functional complications].

In connection with surgical removal of a chordoma in the lumbar region by large excision, followed by 31-month survival of the patient without complications, personal experience of 8 chordomas operated on over the last 20 years is reported. The authors believe their observations show the value of active surgical treatment combined with radiotherapy, which has a palliative effect on the pain and slows the evolution of the tumor. The effectiveness of surgical treatment and radiotherapy leads the authors to challenge the negative attitude generally taken towards the treatment of this condition and to adopt a position of moderate optimism.

Abdominal Muscles↗

PubMed1979

Spinal chordomas.

A series of 54 patients with spinal chordomas were treated at Memorial Sloan-Kettering Cancer Center between 1949 and 1976. Thirty-six lesions were located in the sacrococcygeal region and 18 involved the vertebral column at a higher level. The male to female ratio was 35:19. Vertebral chordomas generally occurred in a younger age group. Our radiological findings suggest that there is marked soft-tissue extension anterior to the vertebral column at the time of initial diagnosis. Eleven of 18 vertebral chordomas and 10 of 36 sacral chordomas were found to have disseminated metastases during their course. Analysis of the various modes of therapy reveals that the median survival for both groups is approximately 6 years. However, the 5-year survival for the sacrococcygeal group was 66% as opposed to 50% for the vertebral group. Radiation therapy produced significant palliation but objective evidence of tumor regression was difficult to assess. Chemotherapy in a small number of patients did not have any effect on the tumor. With the advent of computerized tomography scanning, further studies should be done to document the response of this tumor to radiation therapy.

Adolescent↗

PubMed2026

The molecular landscape of chordoma: Current frontiers from multi-omics to artificial intelligence.

Chordoma is a rare and aggressive malignant bone tumor of the axial skeleton that has historically challenged clinicians due to its complex anatomical locations and a high recurrence rate of up to 85%. This review synthesizes the most recent advances in chordoma research and offers an overview of how multi-omics, advanced immunology, and artificial intelligence are reshaping the treatment paradigm. Central to its pathogenesis is the T-box transcription factor Brachyury, which this review highlights as both the pathognomonic diagnostic marker and the primary therapeutic vulnerability. Cutting-edge innovations targeting this driver include covalent small-molecule binders, targeted protein degradation, and peptide-centric CAR-T cells designed to attack the intracellular oncoprotein. The tumor immune microenvironment is functionally dynamic, and new dimensions in cellular therapy, such as dual-specific CAR constructs and NK-cell platforms, are being engineered to neutralize immunosuppressive factors. Beyond biological insights, the review emphasizes the role of computational biology, specifically how deep-learning and machine-learning models achieve expert-level precision in tumor segmentation and personalized survival forecasting. By integrating genomic, transcriptomic, epigenomic, and proteomic data, multiomics approaches can fully elucidate chordoma subtypes and underlying resistance mechanisms, ultimately paving the way for more precise and personalized therapeutic strategies.

Humans↗

PubMed1979

Chordoma. A clinicopathologic study of metastasis.

Thirty cases of chordoma were reviewed with respect to the incidence of distant metastases. Follow-up information was obtained in 27 (90%), and the incidence of metastases was approximately 30%. The sites of the metastasizing primary tumors were predominantly sacral and vertebral. The sites of the metastases were predominantly skin and bone, although metastases were found in the lungs and lymph nodes. In two of the three patients with dermal metastases, the metastases were present prior to the diagnosis of the primary lesions. All three dermal metastases were initially diagnosed as mixed tumors of the skin, and all three patients had at least four such lesions of the skin. Accurate prediction of which chordomas will eventually metastasize is difficult. Clinically, local aggressiveness and radiotherapy were positively correlated with this ability; histologically, more anaplastic chordomas were more likely to metastasize.

Adolescent↗

PubMed2025

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

The embryonic transcription factor TBXT (brachyury) drives chordoma, a spinal neoplasm without effective drug therapies. TBXT's regulatory network is poorly understood, and strategies to disrupt its activity for therapeutic purposes are lacking. We developed designed ankyrin repeat proteins that block TBXT-DNA binding (T-DARPins). In chordoma cells, T-DARPins reduced cell cycle progression, spheroid formation, and tumor growth in mice and induced signs of senescence and differentiation. Transcriptomic and proteomic analyses identified gene networks involved in cell cycle regulation, embryonic cell identity, and interferon response and revealed features of regulome components, such as susceptibility to pharmacologic inhibition and the fine-tuning of TBXT downstream effectors through IGFBP3. Finally, we found high interferon signaling in chordoma cell lines and patient tumors, which was promoted by TBXT and associated with sensitivity to JAK2 inhibitors. These findings demonstrate the potential of DARPins for probing nuclear proteins to understand the regulatory networks of transcription factor-driven cancers, including entry points for therapies that warrant testing in patients.

Humans↗

PubMed1979

[Chordoma - a review and report of two cases (author's transl)].

Chordomas can be categorized into those of clival, cervical and sacrococcygeal origin. The clival chordomas are primarily discussed in this report. In defining pathological anatomy or histology, a differentiation of benign from malignant chordomas by histological means alone is impossible. A preoperative diagnosis is only possible if tumor grows into the nasopharynx and allows a tissue biopsy to be taken. These tumors are clinically malignant though strict anatomic criteria for malignancy such as nuclear polymorphia or atypia, mitoses and metastases are rarely observed. The therapeutic results of operation and radiation are compared. Each alone fails because of the high recurrence rate of the tumor so that their combination is preferred. Although the prognosis of this disease is poor, one of our patients who was treated with radiation and surgery had a survival time of 14 years.

Adolescent↗

PubMed1979

[Early diagnosis of chordoma by fine-needle aspiration biopsy (author's transl)].

Successful surgical therapy or radiation of chordoma depends on an early morphologic diagnosis. Two cases of chordoma in the sphenooccipital region are reported, in which fine-needle aspiration biopsy was performed. This method is controlled by X-ray. For gaining sufficient material it is superior to surgical biopsy. The cytomorphology of chordoma and its differential diagnosis are discussed.

Adult↗

PubMed1979

[Massive orbital osteolysis by a chordoma. Report of a case and review of the litterature (author's transl)].

Clinical, radiological and anatomical description of sellar chordoma giving, on a sixty seven years old man, an unusually large tumoral exophthalmos, a massive orbito-sphenoidal osteolysis and a chiasmatic syndroma. Discussion stresses the topographic data of chordoma in connection with the embryonal chordal cell rests. Patterns of evolution and neuro-ophthalmologic symptoms of cephalic chordoma are studied with the literature facts. Morphologic criteria, specifical ones and those differential diagnosis, are exactly scheduled.

Aged↗

PubMed1977

[Electronic microscopic study of a sacral chordoma. Characterization of various development stages of the tumor cells].

The electron microscopic study of a sacral chordoma, the cells of which contain variable amounts of glycogen, allowed to characterize different stages in the evolution of the tumoral cells. The young cells contain almost no glycogen ; progressively it becomes more abundant and fills up almost the whole cytoplasm. At the end of the evolution glycogen disappears while the cell becomes vacuolated. This suggests that enzymatic lysis of this polysaccharide could be the cause of the vacuolation which finally produces the physaliferous cell. Comparison between the cells of the chordoma and those of the normal notochord shows some morphological similarities in the development of these cells. These different data may be useful for a classification of the various types of chordomas.

Adult↗

PubMed1979

Subarachnoid dissemination of thoracic chordoma.

Chordoma at the T-9 level, originally manifested by lumbosacral radiculopathy, developed in a 50-year-old man. The patient underwent three operations and radiation therapy for this condition, but ten years later, thoracic myelopathy developed, followed by left facial weakness. On autopsy, extensive dissemination of chordoma was found along the base of the brain and in the leptomeninges of the spinal cord.

Arachnoid↗

PubMed1979

Malignant intracranial chordoma and sarcoma of the clivus in infancy.

Neuroradiological and clinical findings suggesting chordoma of the clivus are described in three children. They presented with torticollis as the primary and predominant symptom. Progressive deterioration with development of slurred speech, signs of intracranial pressure and further cranial nerve involvement led to more extensive neuroradiological investigation. The clinical and neuroradiological findings in these three children were similar; the prognosis was unfavourable for two of the children. The histological diagnosis in two cases was malignant chordoma; in the third case it was undifferentiated sarcoma of the clivus.

Child, Preschool↗

PubMed1979

Craniocervical chordomas.

In a 20 year period seven cases of craniocervical chordoma were seen and treated. Since it is not possible to completely resect these lesions, the aim of treatment has been to debulk the tumor as much as feasible and employ postoperative irridation. Palliation is the most that can be hoped for, and minimal success in delaying the natural history of the tumor has been achieved. Surgery for relief of symptoms secondary to bulky recurrence is indicated for palliation, as is a repeat course of x-ray therapy. Isolated cases of long-term useful survival have followed doses in the range of 10,000 rads or more [19,20]. This therapy is usually given in smaller increments of an initial dose when there is evidence of recurrent tumor or pain [2]. However, whenever the dosage to the spinal cord or the base of the brain exceeds 4,000 rads, there is a concern about possible permanent radiation damage. These patients are difficult to care for late in the course of their disease when multiple cranial nerve paralysis, long tract signs, and intractable pain become progressively severe. Chordomas usually remain localized; however, about 10% metastasize distally. One case is presented of a large retropharyngeal mass with destruction of the second cervical vertebra that recurred locally in 3 months despite usual treatment; distal bony metastases were found 3 years after initial treatment. The histology of the lesion, its recurrence, and distal bony metastases were essentially indistinguishable from the other lesions that remained localized. Short periods of palliation from severe local pain and exquisite tenderness have been achieved by local resection of the involved bones.

Adult↗

PubMed1979

[The chordoma. Description of 2 clinical cases and results of oncolytic chemotherapy].

Only seven hundred cases of chordoma approximatively are reported in medical literature from 1850 to date. We now describe two cases recently observed at our Medical Division, one of sacro-coccigeal chordoma and the other developed from clivus of Blumenbach. Their peculiarities are briefly discussed and compared with a rapid revision of the literature on this argument. The results of antiblastic therapy are presented and its opportunity in addition to Roentgenologic and surgical therapy is discussed.

Adult↗

PubMed1979

Metastatic chordoma.

The authors present two cases of vertebral chordomas with widespread metastases. The literature is reviewed and the metastatic potential of chordomas is analysed.

Adult↗

PubMed1978

[Results of radiation therapy in chordoma: data from seven patients and a review of the literature (author's transl)].

Seven patients with chordoma who were treated in the Universitätsklinik für Nuklearmedizin und Radiotherapie, Zürich, since 1950 are presented. In all the cases, high-dose irradiation resulted in symptom-free survival of many years duration. Five years after the first treatment, all the patients are alive, after ten years about 50% of the patients are alive. Our own experiences as well as the already published cases show that radiotherapy has an important role in the management of chordoma.

Adult↗

PubMed1979

Biochemical studies of the matrix of craniovertebral chordoma and a metastasis.

Glycosaminoglycans of human chordoma and a metastasis were studied. The glycosaminoglycans were derived from the extracellular matrix and consisted of chondroitin 4- and 6-sulphate, keratan sulphate and hyaluronate. The ratio of chondroitin sulphate to keratan sulphate was much lower in the metastasis than in the primary. Proteoglycan extracted with 4M guanidinium chloride and purified by associative density-gradient centrifugation was assessed on Sepharose 2B before and after reduction and alkylation. The result of this experiment suggested that only a small proportion of the proteoglycans were aggregated.

Chordoma↗

PubMed1975

A case of oropharyngeal chordoma.

A case of cervical chordoma without vertebral involvement is presented. The diffculty in histological diagnosis is described. Treatment by radiotherapy and surgery is described and discussed.

Adenoma, Pleomorphic↗

PubMed1976

[Radiological aspects of cervical chordomas (author's transl)].

The radiological findings in two patients with histologically verified cervical chordomas are described and compared with 40 others described in the literature. There is no typical radiographic picture. Our cases showed benign-looking, extensive bone erosions of vertebral bodies and neural arches. In addition, there was destruction of the related discs and a soft tissue mass at the level of the bony lesions.

Cervical Vertebrae↗