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Chordoma is a rare slow-growing malignant neoplasm thought to arise from cellular remnants of the notochord. The evidence for this is the location of the tumors (along the neuraxis), the similar immunohistochemical staining patterns, 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.
Chordomas can arise from bone in the skull base and anywhere along the spine.
The two most common locations are cranially at the clivus and in the sacrum at the bottom of the spine.[1]
In the United States, the annual incidence of chordoma is approximately 1 in one million (300 new patients each year).[2]
There are three histological variants of chordoma: classical (or “conventional”)[3], chondroid and dedifferentiated.
In one study, the 10-year tumor free survival rate for sacral chordoma was 46%.[4] Chondroid chordomas appear to have a more indolent clinical course.
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