Clival tumors are uncommon tumors that come up within the clivus, a portion of bone on the base of the cranium between the occipital and sphenoid bones. This space is surrounded by important constructions and nerves of the brainstem and necessary arteries, reminiscent of the inner carotid arteries. Clival tumors can have an effect on the perform of all these methods, resulting in quite a lot of signs. Clival tumors might be benign or malignant, and remedy choices embody surgical procedures and radiotherapy to take away or cut back the tumor.
What Are Clival Tumors?
Clival tumors might be labeled as both chordomas or chondrosarcomas. Although each sorts of tumors come up within the clival space of the mind, they’ve totally different origins and behaviors.
Clival chordomas are typically benign and slow-growing, however as they develop they’ll trigger vital harm to surrounding areas of the mind resulting from stress and mass impact. Chordomas come up from cells of the notochord, an embryonic precursor to the backbone that develops within the fetus at round three weeks. As the backbone develops, the notochord cells develop into a part of the intervertebral discs within the again. However, in uncommon instances, these notochord remnants can develop into enclosed in bone and start to develop, destroying native tissues and even spreading to different areas of the physique.
Chordomas are uncommon, however chondrosarcomas are even rarer. The origin of this sort of clival tumor is unclear, nevertheless it arises in cartilage within the cranium, which is changed by bone because the physique develops. Chondrosarcomas are typically slow-growing, however they’ll develop into aggressive and have an effect on close by tissues and constructions within the mind. They may metastasize, or unfold to different elements of the physique.
Clival Tumor Symptoms
Symptoms of each chordomas and chondrosarcomas can fluctuate, relying on their location and the encompassing areas of the mind that they have an effect on. Because clival tumors can have an effect on the brainstem, inside carotid arteries and neural pathways on the base of the cranium, signs can embody the next:
- Vision issues
- Coordination issues
- Neck ache
- Facial ache, numbness or paralysis
- Speech issues
- Swallowing issue
- Dizziness and vertigo
- Hearing issue
Symptoms could not seem till the tumor is comparatively massive, and these signs can typically be attributed to different components and situations, which might delay analysis and remedy.
What Causes Clival Tumors?
Although the causes of each chordomas and chondrosarcomas are recognized, it isn’t solely clear what causes these uncommon tumors to develop, and who could also be in danger. People of any age can develop a clival tumor, however males seem to develop clival tumors extra incessantly than ladies. People of European ancestry usually tend to develop these sorts of tumors than these of different ethnicities.
Unlike another sorts of tumors, no recognized environmental or way of life components contribute to creating clival tumors. However, genetic components could play a job as a result of in some instances, a number of members of a household can develop clival tumors. Nearly all people who develop chordomas seem to share a particular genetic trait – a single letter variation in a gene referred to as brachyury that performs a important position within the improvement of the notochord. Chordomas additionally happen at the next price in kids affected by a genetic dysfunction referred to as Tuberous Sclerosis Complex.
How Are Clival Tumors Diagnosed?
Because clival tumors are typically gradual rising, they might not be recognized till they develop into massive sufficient to trigger signs, however the signs they trigger are just like these of many different neurological situations. Diagnosing these kinds of tumors begins with a neurological examination that evaluates features reminiscent of eye motion, coordination, stability and sensations reminiscent of contact and style. Detailed imaging with Magnetic Resonance Imaging (MRI) or Computed Tomography, or CT scans will also be used to differentiate a clival tumor from different sorts of cranium tumors. A biopsy may additionally be wanted to definitively diagnose and kind the clival tumor as a chordoma or chondrosarcoma.
Treating Clival Tumors
Treatment of a clival tumor is determined by components together with the scale and location of the tumor, and the affected person’s age and general well being. The main remedy for each sorts of clival tumors is surgical procedure to take away the tumor fully, or not less than partially. Neurosurgeons could carry out both a craniotomy, which includes eradicating a portion of the cranium to entry the tumor or a transsphenoidal endoscopic surgical procedure, a process which accesses the tumor by way of the nasal cavity. This comparatively new process is much less invasive, doesn’t require a craniotomy, and permits sufferers to get well extra shortly.
Radiation remedy additionally performs an necessary position in treating clival tumors. When a tumor is tough to entry or it may be solely partially eliminated, Gamma Knife Radiosurgery or proton beam radiotherapy could also be used. This method permits neurosurgeons to ship extremely focused radiation to the tumor with out damaging adjoining tissues. Standard chemotherapy regimens are usually not used for treating clival tumors.
After remedy and restoration, sufferers usually obtain common follow-ups with MRI or CT scanning, since clival tumors of each sorts incessantly recur, both on the unique web site or in different elements of the physique. Depending on circumstances, recurrences might be handled with additional surgical procedure, radiation remedy, or each.
Clival chordoma and chondrosarcoma affect about one person in a million, and they’ll happen at any age. However, a spread of remedy choices can relieve signs and assist folks with these very uncommon tumors get again to on a regular basis life.