The term Skull Base Tumors refers to a group of tumors that have a
tendency to grow along various regions of the bottom part of the
skull, mostly on the inside, but occasionally, also on the outside
of the skull. The skull base area separates the base of the brain
from the skull.
Diseases of the skull base are rare, but potentially life threatening
and, were considered inoperable because of their sensitive location,
as well as the possible effects surgery could have on brain function
and complex senses, such as hearing, vision and balance.
Treatment for these lesions is different from other tumors, both in
the surgical approach to their removal and the multi-disciplinary
team of surgeons and physicians required to successfully manage
these problems.
The skull-base, upon which the brain’s undersurface rests, has three
main regions. The Anterior (front) Cranial Fossa is the
region located above the eyes and includes structures such as: the
olfactory bulbs, the nasal cavity, and Cranial Nerves (2, 3, 4 & 6)
that control vision, as well as movement of the eyeballs. The
Middle Cranial Fossa is the region containing the dense,
boney Petrous Ridge, and houses the Internal Carotid Artery, along
with sections of the Cranial Nerves (5 & 7) that control chewing
and facial sensation. The Middle Cranial Fossa also contains the
cavernous sinus, an extremely difficult structure from which to
remove tumors. The Posterior (back) Cranial Fossa is where
the auditory canal and the Cranial Nerves (7 & 8) responsible for
hearing, facial expression and balance are located; the jugular
vein also passes through this region. Of the 24 highly specialized
Cranial Nerves, which control many vital functions of our head
and neck, 18 (Cranial Nerves 3, 4, 5, 6, 7, 8, 9, 10 & 12
two on each side of the skull) originate within this Posterior
Cranial Fossa.
For decades, tumors within the skull-base’s delicate and complicated
bone anatomy were difficult for surgeons to access safely. Patients
with Skull Base Tumors often had a poor prognosis. Advances in
both technology and microsurgical techniques have dramatically
increased surgeons' ability to remove as much of a Skull Base
Tumor as possible.
There are several ways to classify Skull Base Tumors. One common
method is to arrange them by the region that they most often affect.
For example, Anterior Skull Base Tumors may be malignant
or benign. The malignant tumors in this group include tumors
arising in the nasal cavity and paranasal sinuses (such as
Juvenile Angiofibroma, Esthesioneuroblastoma, inverted Papilloma,
Lymphomas, Nasopharyngeal Carcinoma). Other malignant tumors in
this group include Orbital Gliomas and other orbital tumors,
rhabdomyosarcomas, and osteogenic sarcomas. The benign tumors that
occur in the anterior skull base include Meningiomas and Ossifying
Fibromas.
Tumors that are unique to the Skull Base of the Middle Cranial
Fossa are often benign. These tumors include Pituitary
Adenomas, Craniopharyngiomas, Temporal Bone Tumors, Cholesteatomas,
and Enchondromas.
Both benign and malignant tumors can affect the Skull Base of
the Posterior Cranial Fossa. Of the benign tumors Epidermoids,
Dermoids, Chondromas, and Chordomas are the more common while
Chondrosarcoma is the malignant tumor that occurs in this area.
Another way to classify these tumors is by their area of origin.
For example, Primary Tumors arise from the cells of the
anatomical structures in the Skull Base location. Primary Tumors,
arising from inside the Skull such as Meningiomas, Schwannomas,
Chordomas, Glomus Jugulare and Pituitary Tumors, Ossifying Fibromas
and Osteoid Osteomas are often Benign.
Secondary and Metastatic Tumors generally come from outside
the Skull such as tumors arising in the nasal cavity and paranasal
sinuses (Lymphomas, Juvenile Angiofibroma, Esthesioneuroblastoma,
Inverted Papilloma, and Nasopharyngeal Carcinoma). Other malignant
tumors in this group include Orbital Gliomas and other orbital
tumors, Rhabdomyosarcomas, and Osteogenic Sarcomas.
Nasopharyngeal Carcinoma is the most common skull base lesion,
which along with tumors such as Squamous Cell Carcinoma or Adenoid
Cystic Carcinoma and Esthesioneuroblastoma (also called Olfactory
Neuroblastoma), may extend intracranially through the thin bone
of the cribriform plate, which is a part of the Anterior Skull
Base.
The patient's symptoms and signs depend on the structure(s)
impinged upon by the tumor. The skull base is a complex area,
through which traverse the cranial nerves that carry signals to
and from the brain. It is these cranial nerves, as well as the
brainstem, that are at risk to involvement and damage by the
Skull Base Tumor. Although skull base tumors are "outside" of the
brain, they can affect many important functions such as taste,
vision, hearing, swallowing, facial movement and hormonal balance.
Because most tumors grow slowly, symptoms may be present for years
before the correct diagnosis is made.
Tumor location determines the structures that are affected. Tumors
in the Anterior Skull Base may cause dysfunction of the
olfactory or optic nerve, resulting in loss of smell and taste, or
vision in one or both eyes. Tumors in the Middle portion of the
Skull Base may cause eye muscle palsies and loss of sensation
or painful sensations in the face. Symptoms from tumors in the
Posterior Skull Base may include loss of hearing, imbalance,
or difficulty swallowing.
The diagnosis of any Skull Base Tumor must include some form of
Neuroimaging (CT and/or MRI scans). Once the presence of a tumor
of this type is made, patients are generally referred to a center
specializing in the care of these unique problems. Often times,
additional specially designed CT and MRI scans are required by the
treating team of physicians and surgeons. The following
sophisticated investigational techniques are frequently required
by a Skull Base Tumor Team:
CT Scan. Additional vital information regarding the brain
and skull require that thin cuts, along with sagittal and coronal
reconstructions, be obtained in order to determine the extent of the
abnormalities of the bone of the skull and calcifications in the
tumor. 3-D reconstruction of the skull and tumor provide exquisite
details for the treatment team. CT Angiography can be used to
provide some detail of the blood supply to the brain and tumor.
MRI Scan. Studies done, with and without gadolinium (the
"contrast agent"), are essential in order to evaluate the structure
of the tumor and its relationship to vital brain centers. Newer
software programs allow for fine details of the brain's anatomy
to be highlighted and magnified, thus providing vital anatomical
detail. In some instances, Magnetic Resonance Venography, as well
as MR Angiography, may be useful to assess the patency of venous
structures and arteries.
Cerebral Angiography is an important Neuroadiological
procedure, particularly if the tumor encroaches on the carotid
artery, other major intracranial arteries or a major venous sinus.
This can also help assess whether arteries and venous sinuses are
patent and whether pre-operative embolization (obstruction) of
the tumor's blood supply is feasible.
Neuroradiologists, specialists in Neuroimaging interpretation,
are vital members of the treatment team at this point in the
investigation of Skull Base Tumor patients. They use the various
Neuroimaging techniques mentioned above to help the entire
treatment team to understand the anatomical complexity of these
tumors. Since these tumors frequently develop a robust blood
supply, Interventional Neuroradiologists are available with
techniques designed to reduce or eliminate the tumor's blood
supply prior to initiating any definitive surgical therapy. This
is a preparatory step for surgical intervention, which has
helped reduce blood loss during operations.
Other test to assess hearing, balance, phonation and vision may
require specialized technicians and physicians from other
disciplines. This is one area of medicine where patients benefit
from having a team of experts working together to solve these
complex problems.
The goal of therapeutic intervention is to maximize the functional
outcome of the patient, while minimizing their morbidity. Only a
team approach can accomplish this. The multi-disciplinary Skull
Base Tumor Team, which includes specialists from neurosurgery
and many other disciplines, offers patients the best of care for
these very difficult cases. The composition of a Skull Base Team
varies depending upon the nature and location of the tumor. Among
the specialists that may be involved in the diagnosis and treatment
of patients with these lesions are the following:
- Neurosurgeon
- Craniofacial Plastic Surgeon
- Neuro-ophthalmologist
- Interventional Neuroradiology
- Neuro-otologist
- Otorhinolaryngologist
- Plastic Surgeon (including free-flap reconstruction)
- Endocrinologist
- Neurophysiologist
- Radiation Oncologist
- Neuro-Oncologist
- Ophthalmic Plastic Surgeon
- Oral Maxillofacial Surgeon
- Neurological Intensive Care Physician
Many factors should be considered in making a final decision about
the appropriate form of treatment. No single approach is suitable for
every patient. In dealing with these tumors, the Skull Base
Tumor Team acquaints the patient with the benefits, risks and
limitations of each of the available treatment alternatives, so
that the patient and their family can make a well-informed decision
about treatment that they choose.
Treatments can incorporate surgical procedures, medical intervention,
radiation therapy or any combination of these. A tissue diagnosis
is almost always required before medical or radiation therapy is
offered. Chemotherapy can be used as a primary or adjunctive
treatment for many Skull Base Tumors. Radiation therapy or
Stereotactic Radiosurgery can also be used to primarily treat a
Skull Base Tumor, or can be used as adjunctive therapy after
surgical resection.
Most Skull Base Tumors are approached surgically through a
craniotomy, a procedure in which the neurosurgeon makes a
temporary opening in the skull as close as possible to the tumor
site. The basic concept of Skull Base Surgery is to
approach the tumor from the undersurface of the brain and tumor,
or from the side, by removing specific parts of the bone of the
skull base. This permits exposure of the tumor with little or no
retraction of the brain.
In order to fulfill the previously stated requirement to maximize
the functional outcome for the patient, while minimizing the
morbidity, the aggressiveness of the surgical approach must be
adjusted according to the potential impact of the operation on the
patient's quality of life. We recognize that many of these tumors
are benign and often are slow growing. It is frequently in the best
interests of the patient to attempt to remove only part of the
tumor, without adding any major new neurological deficit to an
already difficult neurological situation. In other cases, such
as with many Acoustic Neuromas and Meningiomas, the tumor can be
completely removed with acceptable risks. In other patients, where
the tumor is intertwined with important nerves and arteries, it
is wise to consider removing only that part of the tumor, which
can be removed without damaging the vital arteries and nerves.
The remaining part of the tumor that is intricately involved with
the arteries and nerves can, subsequently, be treated with Focused
Beam Radiation therapy (a specialized form of radiation treatment
called Radiosurgery.) In some cases this Radiation therapy is
preferable to surgery since some tumors cannot be approached,
nor substantial amounts of the tumor removed, without causing a
significant neurological deficit.
There are several highly advanced technologies that are now
available to assist the Skull Base Tumor Team in the management
of these cases. Computer-assisted tumor removal is a surgical
method that uses information obtained from state-of-the-art
computer 3-D imaging techniques, to form computer-generated
models of the tumor. Neurosurgeons, together with the other
Skull Base Tumor Team members, can then plan and simulate the
surgical procedure prior to operating, with the goal of reaching
the brain tumor using the safest and least invasive method
possible. Among the other technological advances are the Minimally
Invasive Microendoscopic and Microneurosurgical techniques, which
incorporate smaller incisions and thus less injury to normal
brain tissue, less blood loss and less post-operative pain
compared to more traditional surgery.
Minimally Invasive approaches allow the Neurosurgeons to
utilize a tiny endoscope with a camera on the end, which is inserted
through the nostril, eyebrow, or other area of the face into the
skull base. The camera provides surgeons with a panoramic view,
and in some cases, allows them to remove the tumor completely.
Endoscopic Brain Surgery is not appropriate for all cases
of Skull Base Tumors although it can be particularly beneficial
in treating certain types of benign tumors.
The Neurosurgeons involved with Neurosurgical Consultants'
Cranial Base Surgery Program specialize in the treatment of
complex tumors, pain syndromes, and congenital defects at the
base of the skull.
Our philosophy is to use Minimally Invasive Microsurgical and/or
Microendoscopic techniques whenever possible. Dr. Lazar and his
colleagues use Endoscopic and Minimally Invasive approaches
to remove a variety of tumor types from various locations including
Transsphenoidal Surgery for Pituitary Tumors, Glabellar approaches
(through an eyebrow incision) for Anterior Skull Base Meningiomas,
Pterional Burr Hole access for the Endoscopic resection of Giant
Arachnoid Cysts of the Middle/Anterior and Posterior Cranial Fossa
as well as for different approaches for Clival lesions.
The Neurosurgical Consultants' Skull Base Surgery Program
particularly focuses on treatment for the following tumors:
- Pituitary Tumors
- Orbital Tumors
- Acoustic Neuromas
- Tumors of the Middle & Posterior Cranial Fossa
(This includes the tumors of the Cerebellopontine Angle, Clivus and
Foramen Magnum)
For additional information or to have your Skull Base Tumor reviewed,
please contact our office.
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This page last edited on 2/20
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