Fifty Years of Experience with Chordomas in Southeast Scotland

O’Neill, Peter; Bell, Blair; Miller, James; Jacobson, I.; Guthrie, W.

doi:10.1227/00006123-198502000-00007

Cited by 71 publications

(30 citation statements)

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“…Consequently, chordomas are most frequently encountered in the sacrum (approximately 50%) or the clivus (35%). 41,73,101 Intracranial chordomas almost invariably involve the clivus. 32,55,56 Within the clivus, the notochord has a sinuous course, thus explaining the diverse origin and projections of cranial base chordomas.…”

mentioning

confidence: 99%

Skull base chordomas: overview of disease, management options, and outcome

Lanzino¹,

As²,

Mb³

et al. 2001

FOC

101

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Cranial base chordomas are locally invasive tumors that, from a midline, clival location, extend in different directions and display various patterns of skull base invasion. Although histologically benign, their invasive nature makes true “oncological” resection virtually impossible to achieve in most cases, despite modern skull base surgical techniques. Moreover, because of the tumor's location and proximity to critical neural and vascular structures, surgery-related morbidity can be significant when an aggressive resection is undertaken. Cytoreductive surgery assumes a critical role in the management of these lesions. The choice of surgical approach and the extent of resection are dependent on several factors: location and extension of the tumor, the surgeon's philosophy and familiarity with a specific approach, and the patient's preexisting clinical status. Proton-beam radiotherapy seems to be effective as an adjunct to surgery in achieving local tumor control. The timing of radiation therapy, however, remains controversial. Gamma knife surgery has been proposed as an adjunctive therapy, but the limited experience and short follow-up periods do not permit formulation of meaningful conclusions at this time. Recurrences are common, although in a subset of patients prolonged disease-free survival is demonstrated.

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mentioning

confidence: 99%

Skull base chordomas: overview of disease, management options, and outcome

Lanzino¹,

As²,

Mb³

et al. 2001

FOC

101

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“…8,24,31) Radiotherapy does not prevent recurrence, or provide tumor regression, 2,3,31) but may increase the disease-free survival. Since chemotherapy is considered to be ineffective, 3,26,31) or needs further investigations, 26) we did not attempt this therapeutic modality.…”

Section: Case Reportmentioning

confidence: 99%

“…Chordoma is a slow-growing malignant tumor originating from notochordal remnants, which accounts for 1-4% of all primary bone malignant tumors, of which 50% occur in sacrococcygeal, 35% in sphenooccipital, and 15% in vertebral locations, 12,26,29,31,32,34) the latter being mostly in the cervical region. 12,18,19,26,31,32,34) Thoracic involvement is 2-5%.…”

Section: Introductionmentioning

confidence: 99%

“…12,18,19,26,31,32,34) Thoracic involvement is 2-5%. 29,31,32,34) Extraosseous chordoma may occur within the sella, paranasal sinuses, larynx, maxilla, and nasopharynx 7) or scapula, 18) as well as ectopic intradural foci in the pons and spinal canal.…”

Section: Introductionmentioning

confidence: 99%

“…9,12,19,34) Vertebral chordomas tend to occur in somewhat younger populations and frequently are more aggressive. 18,26,31) Chordomas affect men more then women at a ratio of 2.7/1 to 1.6/1. 2,3,5,11,18,29,31,34) Female patients are younger, and have longer duration of symptoms, larger tumors, and shorter mean survival.…”

Section: Introductionmentioning

confidence: 99%

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Chordoma of the Thoracic Spine. Case Report.

Topsakal

Bulut

Erol

et al. 2002

Neurol. Med. Chir.(Tokyo)

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A 44-year-old woman presented with a thoracic chordoma with intrathoracic extension manifesting as complaints of lower extremity weakness, hypesthesia below the levels of T5-6, and sphincter incontinence. Almost total resection combined with anterior interbody fusion and stabilization was possible through a left transpleural transthoracic approach. She suffered recurrence after 2 years and was considered inoperable. Biopsy revealed a malignant chordoma with no sarcomatous differentiation. Chordoma is an uncommon malignant bone tumor originating from remnants of the embryonal notochord, occurring mostly along the axial skeleton, at the extremity of the vertebral spine, and is least common in the thoracic region. Differential diagnosis is problematic and biopsy is helpful particularly if considered inoperable. Thoracic chordomas of the malignant type manifest as cord or root compression. Classical malignant chordomas must be distinguished from chondroid, benign, or other types of chordomas, since the biological behavior and clinical features are distinct. However, the differential diagnosis cannot be based on histological examination, but long-term follow up is required. Most patients have extradural and intraspinal tissue extension at the time of diagnosis, which makes complete resection impossible. Aggressive surgery without violation of surgical borders is the best choice in the treatment of thoracic chordoma. Thoracic chordoma is a recurring neoplasm and is prone to dissemination and sarcomatous differentiation despite its slow-growing nature.

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Dedifferentiated chordoma: Response to aggressive chemotherapy in two cases

Fleming

Heimann

Stephens

et al. 1993

Cancer

119

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Background. Dedifferentiated chordoma is an unusual and aggressive variant of chordoma which is likely to metastasize. Few reports exist of treatment of these tumors with chemotherapy. Methods. In 1988, two patients with dedifferentiated sacral chordomas were seen at the University of Chicago Hospitals. Both developed metastatic disease less than a year after sacral resection and radiation therapy. These patients' diagnoses, courses, and treatments were reviewed along with the literature on chemotherapy in both conventional and dedifferentiated chordomas. Results. Both patients obtained complete remissions, one to a six‐drug regimen and the other to ifosfamide. Conclusions. A trial of reasonably aggressive chemotherapy is warranted in patients with metastatic dedifferentiated chordoma. The optimum regimen is unclear, but agents active in high‐grade sarcomas are logical choices.

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Fifty Years of Experience with Chordomas in Southeast Scotland

Cited by 71 publications

References 0 publications

Skull base chordomas: overview of disease, management options, and outcome

Skull base chordomas: overview of disease, management options, and outcome

Chordoma of the Thoracic Spine. Case Report.

Dedifferentiated chordoma: Response to aggressive chemotherapy in two cases

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