Chordomas

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About Chordomas Tumors

Overview

  • Chordomas are rare tumors of the remnant of the primitive notochord, which normally differentiates during fetal development into an elastic, pulpy mass within the cartilage between the spinal disks.
  • Chordomas occur most commonly in the lower spine and in the skull base area. Approximately 40 percent of chordomas arisewithin the skull base, and 0.1 percent of intracranial tumors are chordomas.

Symptoms

  • Despite the typically large size of these tumors, patients usually have minimal symptoms because of the slow growth rate. Most patients experience headaches and double vision.
  • Less common symptoms include visual loss, hearing loss, difficulty swallowing, hoarse voice, facial numbness, incoordination, motor weakness and memory disturbance.

Diagnosis

  • These tumors are diagnosed by magnetic resonance imaging (MRI) and Computed Tomography (CT) scans, which will clearly show the extent of damage from the tumor.

Treatment

  • Initial treatment is usually with surgery, either through an opening made in the skull or, in a minority of cases, by the transsphenoidal route.
  • Because chordomas invade the bone and covering of the skull base, complete surgical removal is difficult and continued tumor growth is common.
  • Extensive surgery can improve long-term survival but aggressive tumor removal can be associated with significant surgical complications, including neurological problems.
  • Stereotactic radiosurgery is often used to treat residual or recurrent tumor.
  • Most recurrent or residual tumors are best treated with proton beam radiation or with stereotactic radiosurgery. External beam irradiation of chordomas is effective only occasionally

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The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the Neuro-ICU attending physician and team members direct your family member's care while in the ICU. The Neuro-ICU team consists of the bedside nurses, nurse practitioners, physicians in specialty training (Fellows) and attending physicians. UCLA Neuro ICU Family Guide