chordoma
MONDO:0008978
Chordomas are rare malignant tumors arising from embryonic remnants of the notochord in axial skeleton.
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Chordoma
Chordoma is a rare slow-growing neoplasm (cancer) that arises from cellular remnants of the notochord in the bones of the skull base and spine. The evidence for the notochordal origin of chordoma is the location of the tumors (along the neuraxis), the similar immunohistochemical staining patterns, expression of brachyury, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the notochord regresses during fetal life. In layman's terms, chordoma is a type of bone cancer, and is classified as a sarcoma. Chordomas are sometimes mistakenly referred to as brain, brainstem, or spinal-cord tumors due to their location near those critical structures, but they are not derived from nervous tissue.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Clinical trials
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Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.