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INTRAMEDULLARY TUMOR OVERVIEW

General Comments

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.

Hemangioblastomas 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 indolent.

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 article Spinal Lipomas 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.

The diagnosis of Lipomyelomeningocele is usually made early in childhood when a peculiar skin lesion or fatty mass is observed. IT IS IMPORTANT FOR PEDIATRICIANS, NURSE PRACTITIONERS AND GENERAL PHYSICIANS TO RECOGNIZE THAT THE PRESENCE OF A "SKIN MARKER" OVERLYING A CHILD'S SPINE MAY INDICATE AN UNDERLYING SPINAL DYSRAPHIC CONDITION.

Unfortunately this problem may not be made until adulthood when back pain or a progressive neurological deficit calls attention to the problem.


SYMPTOMS

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.

DIAGNOSIS

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.

Figure 1: MRI Scan (Sagittal View-Gadolinium enhanced)

Extensive Cervical Spinal Cord "Mixed Glioma" with Syrinx in the Medulla

The Syrinx (Left-curved Arrow) at the Cervico-medullary Junction causes considerable displacement and compression of the surrounding Neural Structures.

The Tumor (Horizontal Arrow) extensively infiltrates the Spinal Cord.


TREATMENT

Surgery

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 well-circumscribed Astrocytomas.

Figure 2A (Left): Post-myelographic CT Scan (Axial View) L5 Level Tethered Spinal Cord with Filum Terminale Lipoma, Bifid Spine (Horizontal Bi-directional Arrow) in a 38 year old male with Urinary incontinence and Leg Pain. At this level of the Spinal Canal one would not find Intradural Nerve Roots of the diameter represented by the images demonstrated here (Oblique Arrows).(See Figures 3 & 4 for Operative Photos)

Figure 2B (Right): Post-myelographic CT Scan (Axial View-Same Patient) Tethered Spinal Cord with Filum Terminale Lipoma and Bifid Spine. The point of "Tethering" is indicated by the centrally located posterior intradural structure (Vertical Arrow.) The posterior Spinous Process and Lamina are also malformed (Right-angled Arrow.)

Figure 3: Operative Photo (Same Patient). L4, L5 and Sacral Laminectomies were performed to gain access to the Filum Terminale Lipoma with Tethered Spinal Cord Syndrome. In this photo the Dura has been opened, the Lipoma has been transected and the Tether has been relieved (Vertical Arrow indicates the Distal portion of the Lipoma.) The Filum Terminale is markedly expanded by the Lipoma (Down-curved Arrow.)

Figure 4: Operative Photo (Same Patient.) The Distal portion of the Lipoma (Left Curved Arrow) has been resected and the Tethering of the Spinal Cord has been relieved. The Sacral Nerve Roots (Left Sided) are seen in the Extra-dural Space (Horizontal Arrows) after extensive Decompressive Foramenotomies were performed. The Intra-dural segment of the Sacral Nerve Root is seen exiting the Dura (Vertical Arrow) and can be followed to its Extra-dural component.

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 Spinal Cord.

Radiation Therapy

Radiation therapy may also have a role in the management of persistent, recurrent, or progressive Low-grade Astrocytomas and Ependymomas.

Stereotactic Radiosurgery

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.

Chemotherapy

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 Neuro-oncologists 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 "Virtual Trials" website.

Rehabilitation

Rehabilitation programs are an important component of the multidisciplinary care of patients with Spinal Cord Tumors.

PROGNOSIS

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.

DISCLAIMER: Every effort has been made by the author(s) to provide accurate and up-to-date information. However, the medical knowledge base is dynamic and errors can occur. By using the information contained herein, the viewer willingly assumes all risks in connection with such use. Neither the author nor Neurosurgical Consultants, p.a. shall be held responsible for errors, omissions in information herein nor liable for any special, consequential, or exemplary damages resulting, in whole or in part, from any viewer(s)' use of or reliance upon, this material.

CLINICAL DISCLAIMER: Clinical information is provided for educational purposes and not as a medical or professional service. Person(s) who are not medical professionals should have clinical information reviewed and interpreted or applied only by the appropriate health professional(s).


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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.