Rare-30. Anaplastic Pleomorphic Xanthoastrocytoma With Leptomeningeal Dissemination Responsive to Braf Inhibition and Bevacizumab

Abstract

NEURO-ONCOLOGY • NOVEMBER 2017 were alive at the time of analysis. 2 patients identified as Asian and 7 as White. The MiB labeling index (MiB) ranged from ≤2 to 20%. All underwent surgical resection and GTR was achieved in 5. Of those patients with GTR, 3 (MiB 3-5, 5 and 20%) received adjuvant radiotherapy (RT). A single patient with GTR and elevated MiB of 5% did not receive RT: this patient is progression-free at 38 months and is the only patient with a KPS of 100. There was a single case of recurrence in the patient with highest MiB (20%) treated with GTR, RT, and concurrent temozolomide. This patient is alive >10 years from diagnosis and 5.2 years after recurrence. A majority that received RT (n=6), were referred for neuropsychological evaluation with 4 patients requiring use of methylphenidate. CONCLUSIONS: This tumor has a favorable prognosis even with elevated MiB and in the setting of recurrence. Treatment for recurrence may include GTR and conformal RT with temozolomide. Further research on RT sparing treatments in the adjuvant setting is warranted. Referral for neuropsychological evaluation and symptomatic treatment with methylphenidate should be considered in patients who receive RT. RARE-28. MALIGNANT TRANSFORMATION OF A LOW-GRADE MIDLINE GLIOMA WITH CONCOMITANT BRAFV600E AND H3.3K27 MUTATIONS

Pleomorphic xanthoastrocytoma: a brief review

“…More than half were noted to have a confirmed clinical benefit [63]. The addition of bevacizumab to this regimen at time of progression may prolong survival [64]. The utilization of BRAF inhibitor in conjunction with tumor-treating fields (TTFields) has also been reported [65].…”