Outcomes

The histologic subtype may be the most significant predictor of prognosis after treatment in cervicomedullary tumors.10 Bricola reports in his series of children and adults with cervi-comedullary tumors that 6 of 12 patients (one ependymoma, five benign astrocytomas) made excellent neurologic recoveries, although the remainder (four malignant gliomas, one angioreticuloma, and one lipoma) showed no appreciable improvement.2 In Epstein's series,7 there were 24 patients with cervicomedullary gliomas. Sixteen had grade I or II astrocytomas, and four had gangliogliomas. All 16 patients were alive and neurologically improved 6 to 60 months postoperatively. All four patients with grade III or IV astrocytomas died 9 to 12 months postoperatively from tumor progression.

Although radical surgery offers the possibility of long-term clinical remission,25 there are significant complications. Reported postoperative deficits can include severe quadripare-sis, respiratory difficulties resulting in ventilatory dependency, and swallowing difficulties that require gastrostomy.5,19 Mortality from meningitis and sepsis, tumor progression,7 aspiration pneumonia,5 aspiration with pulmonary insufficiency,23 and apnea and respiratory arrest25 has been reported. The immediate postoperative course has been related to the preoperative neurologic status, because patients who are severely disabled before surgery recover very slowly, whereas those who are less impaired improve more quickly.5 Given the increased risk of respiratory and airway compromise in these patients, recommendations have been made for tracheostomy or prolonged intubation for up to 7 days postoperatively.23 Long-term postoperative complications include cervical kyphosis in three out of nine patients after suboccipital craniotomy and cervical laminectomy requiring surgical fusion in two25 (Figure 84-7).

The therapeutic series are too small to make definite conclusions regarding the various treatment strategies. In the

Figure 84-7

Figure 84-7

A, Postoperative sagittal image taken from the same case as depicted in Figure 84-4. Note radical resection of tumor but the development of cervical kyphosis. B, Occipital-cervical instability necessitated a fusion procedure using an occipital to C3 fusion construct with a Luque rectangle, sublaminar and suboccipital wires, and bone grafting.

Young Poussaint et al series,25 patients underwent surgery alone, surgery with radiation, and surgery with chemotherapy and radiation. In their six patients who had surgery alone, subtotal resection was performed in four, biopsy only in one, and biopsy followed 1 month later by subtotal resection for tumor in one. Of these patients, four had stable residual disease, and two had no visible tumor. All are alive with a mean follow-up of 3.9 years (range 0.2 to 11 years). In four patients who underwent surgery followed by radiation, three underwent subtotal resection and one underwent biopsy. Progression of the disease occurred in three and the fourth died of respiratory arrest with stable residual tumor. With radiation treatment and mean follow-up of 8.2 years (range 4.3 to 10 years) of the remaining three patients, two have stable residual tumor, and one has no tumor. One patient had subtotal resection with increasing tumor enhancement found 1 month later. Because of the young age of the patient (1 year), the patient was initially treated with carboplatin and vincristine. Although decreased tumor enhancement was initially observed, 1.5 years later the patient developed a metastatic nodule in the brain consistent with cerebrospinal fluid seeding. When the original tumor was found to increase in enhancement 2 months later, the patient underwent radiation therapy. At 2-year follow-up review, the patient was reported to have had interval tumor resolution with stable clinical findings.

With respect to tumor progression, the majority were related to local tumor growth rather than metastatic disease.19,25 Robertson et al19 report that 4-year actuarial progression-free survival rates and total survival rates were 70% and 100%, respectively, in patients after first surgery for a newly diagnosed tumor. Progression-free and total survival rates were poorer, 41% and 62%, respectively, in patients who had received prior radiation therapy for their tumor. Median follow-up in this series was 4 years.19 In the three patients with newly diagnosed tumors with progression, treatment consisted of repeated resection with adjuvant radiation therapy. The histopathology of low-grade astrocytoma in two and anaplastic astrocytoma in the other was not found to have changed. In three of six patients who had progressed after prior radiation treatment and surgery, one was stable after a second resection, and two died. From this study, it was concluded that patients with prior radiation treatment or severe preoperative deficits of bulbar function (swallowing, speaking, breathing) and ambulation had the highest morbidity. Neurologic deterioration with significant disability has also been reported to occur as late as 1 to 3 years following radiation therapy.5

Others, however, find no correlation with radiation therapy or preoperative symptomatology. Weiner et al24 report that no statistical correlation was found between 5-year progressionfree survival, total survival, or neurologic outcome and age, gender, radiographic tumor appearance, prior surgery, radiation therapy, chemotherapy, preoperative symptomatology (spinal cord versus medullary), extent of resection, or postoperative adjuvant therapy.

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