Chordoma
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| Chordoma Classification and external resources | |
| ICD-O: | M9370/3 |
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| OMIM | 215400 |
| DiseasesDB | 31483 |
| eMedicine | med/2992 radio/169 orthoped/49 |
| MeSH | D002817 |
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US National Guidelines Clearinghouse on Chordoma
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Overview
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 anywhere along the neuraxis. The two most common locations are cranially at the clivus and at the sacrum.
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
In one study, the 10-year tumor free survival rate for sacral chordoma was 46%.[1] Chondroid chordomas appear to have a more indolent clinical course.
Aggressive surgical resection followed by radiation therapy offers the best chance of long-term control.
There are no drugs currently approved to treat chordoma, however chordoma has been shown to have a limited response to the PDGFR inhibitor Imatinib.[1]
References
External links
- Chordoma Foundation
- Chordoma Support Group
- Images of Chordoma - mostly radiological (CT and MRI scans), one autopsy image
Nervous tissue tumors (ICD-O 9350-9589) | |
|---|---|
| Miscellaneous tumors (9350-9379) | Craniopharyngioma - Pinealoma - Chordoma |
| Glioma (9380-9489) | Gliomatosis cerebri - Oligoastrocytoma - Choroid plexus papilloma - Ependymoma - Astrocytoma (Pilocytic astrocytoma, Glioblastoma multiforme) - Dysembryoplastic neuroepithelial tumour - Oligodendroglioma - Medulloblastoma - Primitive neuroectodermal tumor |
| Neuroepitheliomatous (9490-9529) | Ganglioneuroma - Neuroblastoma - Atypical teratoid rhabdoid tumor - Retinoblastoma - Esthesioneuroblastoma |
| Meningiomas (9530-9539) | Meningioma |
| Nerve sheath tumor (9540-9579) | Neurofibroma (Neurofibrosarcoma, Neurofibromatosis) - Schwannoma - Neurinoma - Acoustic neuroma - Neuroma |
| see also brain tumors (though not all brain tumors are of nervous tissue) | |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
