These Tumors usually arise from cells within the Spinal
Cord. The most common are those originating
from the Spinal Cord's supporting cells ("glia") and therefore
lie within the substance of the Spinal Cord itself. (Please
refer to our section on
"Brain Tumors" for
a discussion of the Glial series of tumors.) Astrocytomas and
Ependymomas account for the majority of these tumors and occur
with about equal frequency.
are less common and sometimes occur in conjunction with
Von Hippel Lindau Disease
(a disease in which patients are prone to developing cysts in the
kidney and other organs). These Intramedullary Tumors occur most
often in the Cervical Spinal Cord and are often quite
Lipomas (Fat Cell Tumors) are unusual benign
tumors. There are a couple of varieties that occur as
Intramedullary Spinal Cord lesions. (For a thorough
review please consider consulting the comprehensive
by Jeffrey P. Blount, M.D., and Scott Elton, M.D.)
A. Lipoma of the Terminal Filum is a truly occult
lesion; therefore the true incidence of "fatty
filum" is unknown. In modern times,
more of these "lesions" are incidentally
identified by virtue of the fact that MRI Scans
are done to investigate other conditions. According to authors
Blount & Elton, Lipomas limited to the Filum Terminale
"represented 13 to 26% of lesions accounting for tethered
cord." The "Tight Filum Syndrome" is one of the
causes of a "Tethered Spinal Cord".
In these cases an abnormally enlarged Filum Terminale
prevents the normal ascent of the Spinal Cord within the
Lumbar Spinal Canal during normal development. (See Figures 2A &
2B) The medical literature on this subject claims that
"greater than 90% of cases in which this diagnosis is made demonstrate
adipose (fat) cells in the Filum Terminale".
B. Lipomyelomeningocele (Lipoma of the Conus Medullaris)
is the most common "fatty mass" occurring along or within the
Spine. Lipomyelomeningocele is a form of Occult Spinal
Dysraphism in which a subcutaneous fibro-fatty mass extends
through the fascia in the Thoraco-lumbar region to cause a
"defect" in the Spinal Lamina to displace the Dura as it
infiltrates and "tethers" the Spinal Cord.
Lipomas of the Conus Medullaris occur in 1 in 4000 births
in the United States with females being affected slightly more
often than males.
INTRAMEDULLARY SPINAL CORD TUMORS can be DIFFICULT TO
DIAGNOSE EARLY in the course of the disease process, since the
CLINICAL FEATURES OF INTRAMEDULLARY SPINAL CORD TUMORS ARE QUITE
VARIABLE. Early symptoms are usually nonspecific and may only subtly
progress. SYMPTOM DURATION (FOR THE LESS AGGRESSIVE TUMOR VARIETIES)
BEFORE DIANOSIS is often 2 to 3 years, whereas the MORE
MALIGNANT PRIMARY TUMORS AND METASTATIC NEOPLASMS cause problems
within a much shorter course, usually in several weeks
to a few months.
PAIN is the most common presenting symptom of Intramedullary
Spinal Cord Tumors in adults. The pain typically
localizes to the level of the Tumor. Sensory (numbness
and/or tingling) or motor (weakness and spasticity) complaints
are the initial symptoms in up to one third of
patients. Upper extremity symptoms predominate in cases of Cervical
Tumors. One sided (or asymmetrical) involvement is common.
Thoracic Spinal Cord Tumors produce spasticity and sensory
changes. Numbness is a common complaint and
usually begins in the more peripheral locations of the lower
extremities. Tumors of the Lumbar region and Conus Medullaris
present with back and leg pain. Bladder and bowel dysfunction tends
to occur early.
A careful history and physical examination is the first step in
diagnosis. MRI scanning without and with contrast agent (Gadolinium)
is the PRIMARY METHOD TO DIAGNOSE THESE TUMORS and generally
offers excellent anatomical details regarding the position of the
Tumor and its relationship to the Spinal Cord.
Surgical removal is the most effective and primary
treatment for most Intramedullary Spinal Cord Tumors. The
role of surgery is determined primarily by the
nature of the interface between the Tumor and the Spinal Cord.
Determination of the resectability of an Intramedullary Spinal Cord
Tumor is best made by direct intraoperative inspection of the
Tumor-Spinal Cord interface.
Some "localized" tumors may be accessible using
Minimally Invasive Microsurgical Techniques.
A. Intramedullary Tumors That CAN Be Totally Removed.
In cases of benign tumor, our strong preference favors
Minimally Invasive Microsurgical Gross Total Removal particularly
if a clear demarcation/interface exists between the Tumor and the
Spinal Cord. Surgical removal alone provides long-term tumor
control or cure for Hemangioblastomas, almost all
Ependymomas, Filum Terminale Lipomas and some
B. Intramedullary Tumors That CANNOT Be Totally Resected.
Some Glial Tumors that cannot be totally resected or are
recognized to be more aggressive (Anaplastic
Astrocytoma/Glioblastoma, etc.) will require Post-operative
Radiation Therapy and possibly Chemotherapy as well.
Lipomyelomeningoceles cannot usually be resected due to the fact
that functional neural tissue is intimately interlaced with the
Lipoma. The only supportable indication for surgery in these patients
is Neurological Symptoms relating to demonstrable Tethering of the
Radiation therapy may also have a role in the management
of persistent, recurrent, or progressive Low-grade Astrocytomas
Recent reports have described the use of Stereotactic Radiosurgery
for Metastatic Spinal Tumors. This exceptionally accurate
Radiation Therapy system should be considered in the management
of Intradural Extramedullary as well as Intramedullary
Spinal Cord Tumors.
Modern Chemotherapy has a role for certain progressive Spinal
Cord tumors. Chemotherapy is the third arm of this
comprehensive effort to prolong and maintain a high quality of
life. Traditional management has been to use "standard" forms
of Chemotherapy. Currently there are some unconventional
chemotherapeutic alternatives that offer considerable hope for improved
quality and length of survival. One of our
has utilized these newer medications such as
Temodar (temozolamide), Avastin (bevacizumab, an
anti-angiogenesis agent) and CPT-11, either alone (or more
commonly) in combination or with other drugs, to produce
encouraging results. Additional information regarding these
treatments is available at the
Rehabilitation programs are an important component of the
multidisciplinary care of patients with Spinal Cord
Long-term outcome and risk of recurrence depend primarily on Tumor
histology, the completeness of the original resection and
responsiveness to Radiation and /or Chemotherapy.
The current treatment of Spinal Cord High-grade Astrocytomas,
which includes surgical debulking, Radiation Therapy and
Chemotherapy, is usually inadequate.
For the less aggressive Tumors, long-term survival is common
although Neurological deficits consequent to the Tumor's effect can be
quite severe and disabling.
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This page last edited on 2/19