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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.
In the United States, the annual incidence of chordoma is approximately 1 in one million.
There are three histological variants of chordoma: classical, chondroid and dedifferentiated. The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as physaliferous (resembling a spider's web). Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.
Prognosis and treatment
Aggressive surgical resection followed by radiation therapy offers the best chance of long-term control.
|This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Chordoma". A list of authors is available in Wikipedia.|