Chordomas are often considered "benign" tumors that are amenable to surgical resection alone. However, they can be invasive and can metastasize, and, without complete resection, they can produce local regrowth. They are considered embryonal tumors and are remnants of the notochord. The primary histological feature is the presence of large mucin-containing physaliferous cells. Areas of hemorrhage and calcification may also be seen. Chordomas may degenerate to a more malignant histological appearance, the chondrosarcoma. Chordomas originate in essentially two locations, the clivus or sphenoid region and the sacrococcygeal region, although they can arise in other areas as well, including infiltration into the facial structures. They are relatively rare and account for less than 1 percent of primary CNS tumors. They usually occur at mid-life and predominate in men. Men who have smaller non-necrotic tumors seem to have a better overall outcome. y

Clinical presentations of patients with clivus or cervical region tumors include headache, oculomotor nerve abnormalities, or other cranial nerve dysfunction, especially in the lower motor nerves. Tumors in the sacrococcygeal region present with symptoms similar to those typical of cauda equina or conus medullaris disruption. Radiological evaluation should include both CT for examination of bone abnormalities and MRI to evaluate soft tissue destruction. These tumors are extra-axial, and displacement of brain stem or other structures may be seen. Nerves exiting from the nervous system may become entrapped in the tumor. Chordomas can be confused with craniopharyngiomas, meningiomas, metastatic carcinoma, or possibly adenoma. They show variable enhancement with contrast dye. Angiography may be necessary to define the arterial structures prior to surgery. The differential diagnosis for these tumors includes the malignant variant, chondrosarcoma, and other lesions known to occur in this region such as meningiomas or schwannomas, metastatic tumors such as carcinomas, or, remotely, craniopharyngiomas.

Definitive surgery for diagnosis and maximum tumor removal is imperative. Patients should be referred to large centers staffed by neurosurgeons who are adept at skull-base surgery. Tumors usually exhibit regrowth at the site of the previous resection. Radiotherapy, including directed stereotactic radiotherapy, is improving overall survival; however, radiotherapy should be reserved for patients who show evidence of tumor after surgery.

In a recent report of 60 patients with chordoma or chondrosarcoma, overall regrowth-free survival at 5 years was found in 84 percent of patients with near-total resection and in 64 percent of patients with partial resection. y Extent of surgical resection was the greatest predictor of overall survival. Importantly, extent of surgical resection did lead to some decrease in patient performance postoperatively, but with rehabilitation, most patients regained their level of preoperative performance in about 6 months.

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