Prognostic Factors and Therapeutic Outcomes in 22 Patients with Pleomorphic Xanthoastrocytoma

OBJECTIVE: Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known. METHODS: The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA. RESULTS: The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients. CONCLUSION: The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.

Primary Spinal Cord Astrocytoma Presenting as Intracranial Hypertension: A Case Report

Increased intracranial pressure (IICP) is rarely seen in association with primary spinal tumors. We describe a 58-year-old man who was diagnosed with a primary spinal cord astrocytoma, who first presented with hypesthesia, followed by intracranial hypertension, papilledema and blurred vision. On first admission, he presented with hypesthesia but without paraparesis, headache or blurred vision. Spinal MRI showed a relatively well-enhanced solid mass with a cystic portion at the cervico-thoracic level, shown histologically to be a grade I pilocytic astrocytoma. After gross total resection of the tumor, the patient had no significant neurological changes. Nine months later, the patient was admitted with headache, blurred vision and paraparesis. An ophthalmologic examination showed papilledema and lumbar tapping revealed IICP. A spinal MRI showed recurrence of the tumor which was found to be a glioblastoma after reexplorative debulking surgery. After resection, his headaches and blurred vision improved, but his paraparesis did not. These findings show that a primary spinal cord astrocytoma may cause IICP.