Chordoma
| Chordoma | |
|---|---|
 |
|
| MRI of extensive clival chordoma in 17-year-old male patient, axial view. Tumor in the nasopharynx extending from nasal cavity to brainstem posteriorly is clearly visible. | |
| Classification and external resources | |
| ICD-O | M9370/3 |
| OMIM | 215400 |
| DiseasesDB | 31483 |
| eMedicine | med/2992 radio/169 orthoped/49 |
| MeSH | D002817 |
Chordoma is a rare slow-growing neoplasm thought to arise from cellular remnants of the . 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 regresses during fetal life.
There are three histological variants of chordoma: classical (or "conventional"), chondroid and dedifferentiated.
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.
A small number of families have been reported in which multiple relatives have been affected by chordoma. In four of these families duplication of the brachyury gene was found to be responsible for causing chordoma.
A possible association with tuberous sclerosis complex (TSC1 or TSC2) has been suggested.
In 2015 the first consensus guidelines for the diagnosis and treatment of chordoma were published in the Lancet Oncology.
In one study, the 10-year tumor free survival rate for sacral chordoma was 46%. Chondroid chordomas appear to have a more indolent clinical course.
In most cases, complete surgical resection followed by radiation therapy offers the best chance of long-term control. Incomplete resection of the primary tumor makes controlling the disease more difficult and increases the odds of recurrence. The decision whether complete or incomplete surgery should be performed primarily depends on the anatomical location of the tumor and its proximity to vital parts of the central nervous system.
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Wikipedia