Observing a brain scan

Chordoma

Endoscopic endonasal surgery is the optimal initial treatment for most Clival Chordomas

Clival chordomas are locally invasive tumors of the midline skull base.

Clival chordomas are ideally treated with maximal safe surgical removal followed by focused radiotherapy. Fortunately, the majority of clival chordomas can be removed via the nose using an endoscopic endonasal approach.  Occasionally, these tumors require multiple keyhole operations including the retrosigmoid and eyebrow craniotomy approaches.

At PNI, we have one of the world’s largest experiences with endonasal surgery for clival chordomas including patients who have had prior surgery and radiation. By incorporating cutting edge technology and instrumentation with proven surgical experience, our highly collaborative team provides safe, less invasive and effective chordoma surgery.

Given that many chordomas can be resistant to both surgery and radiosurgery, tumor genomic profiling is routinely performed in our chordoma patients to find alternative targeted therapies. We also have a new clinical trial open with pemetrexed (Alimpta) for patients with chordoma which we can offer for some patients.

New Chordoma Clinical Trail

We now routinely perform tumor genetic profiling to assess for tumor markers that can be targeted using newly available chemotherapies or immunotherapies, and have a new clinical trial open with pemetrexed (Alimpta).

Watch the video below to learn more about the clinical trial.

Overview

Chordoma are locally invasive slow-growing malignant tumors that arise from the remnant of the primitive notochord. They occur most commonly in the skull base (clivus) and lower spine. Approximately 40% of chordomas and chondrosarcomas arise in the clivus (directly below the sella turcica and pituitary gland).

Chordomas can behave quite aggressively and are often extensively invasive into surrounding skull base structures. A small subset of patients (~10%) may develop metastases to distant sites beyond their site of origin.

Symptoms

The most common presenting symptom of a clival chordoma is double vision.

Less common symptoms may include:

  • Facial numbness or tingling
  • Loss of vision
  • Hearing loss
  • Difficulty swallowing
  • Coordination
  • Motor weakness
  • Nasal congestion
  • Pituitary gland dysfunction
  • Headache

Diagnosis

These skull base tumors are best diagnosed by MRI and CT scans which will clearly show the extent of tumor and bony destruction.

Focused MRIs of the pituitary region, sinuses, temporal bones or internal auditory canals may be indicated to obtain better anatomical detail of a chordoma.

Other tests may also be needed prior to surgery such as angiography (typically now performed as a CT angiogram or an MR angiogram), visual field tests, an audiogram or pituitary hormonal tests.

Treatment

The initial treatment for a clival chordoma is maximal safe surgical removal.

Given their midline location, the great majority of clival chordomas and chondrosarcomas are best removed via an endoscopic endonasal approach.

However, some extensive and/or laterally placed chordomas may require different skull base approaches. Because chordomas typically invade the bone and dura of the skull base as well as cavernous sinus area, complete surgical resection is often not possible and continued growth of a residual tumor is common.

Extensive surgery can certainly improve long term survival but over-aggressive tumor removal can be associated with significant neurological complications.

After maximal safe removal, most chordoma patients will require Stereotactic Radiotherapy (SRT), stereotactic radiosurgery (SRS) or proton beam radiation. Chemotherapy is generally ineffective for treating chordomas although clinical trials of some experimental chemotherapies and immunotherapies are available.

Written and reviewed by:
We are a highly specialized team of medical professionals with extensive neurological and cranial disorder knowledge, expertise and writing experience.
Last updated: May 14, 2020