Although rare, spinal cord tumors continue to carry a grave prognosis for patients. They are difficult to resect and treat. Both the tumor itself and the treatment often lead to profound physical and neurological disability due to spinal cord damage. Spinal cord tumors represent about 7 percent of all primary tumors of the CNS and are seen more commonly in children, in whom they represent up to a fourth of all intra-axial tumors by location. The most common histological type is that of the glioma histology, especially astrocytomas and ependymomas. Their frequency is undoubtedly related to the percentage of total tissue of the spinal cord involved.
Often patients are brought to medical attention because of weakness of the legs, loss of bowel or bladder control, back pain, or, rarely, loss of sensation. In children, these symptoms may be confused with "regression" of the child's development, "growth pains," or muscle disease. Often a child's lesion is discovered incidentally during evaluation for a minor injury such as a sledding accident, when studies are performed to calm the fears of worried parents. Cervical or foramen magnum lesions may present with torticollis or nuchal rigidity.
Most patients are evaluated by MRI imaging, which demonstrates an enlarged or thickened cord. Frequently, the spinal canal may be enlarged around the area of the slowly expanding tumor. Once the tumor has been identified, the entire neuroaxis should be studied for any other areas of involvement. Although a tissue diagnosis may be suggested by the tumor's location and radiographic pattern, pathological confirmation is essential prior to treatment. All patients should be evaluated by a neurosurgeon for consideration of biopsy or evacuation of the lesion. Preoperative and postoperative testing with evoked potentials can be performed to monitor disease evolution. Because of the location, patients are at a significant risk of morbidity from the tumor and any treatment for it.
Treatment of these tumors includes surgical evacuation when possible, followed by radiotherapy to a malignant lesion and the surrounding cord. Low-grade tumors may be monitored closely for evidence of recurrence or malignant degeneration. In children, consideration of a delay in radiotherapy is essential to prevent unwanted side effects due to the treatment. Although chemotherapy has not been systematically studied in these patients, one would intuitively expect that agents with known effects on supratentorial tumors would also be effective on spinal tumors with a similar histology. Patients with spinal column defects, such as scoliosis, that are related to the tumor or treatment can be treated appropriately with surgery (if necessary) and physical therapy.
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