Treatment of Recurrent Anaplastic Pleomorphic Xanthoastrocytoma in a 33 Female Previously Diagnosed with WHO Grade I Ganglioglioma

Purpose: Anaplastic Pleomorphic Xanthoastrocytomas are rare neoplasms accounting for approximately 1% of primary brain tumors and generally present in the 2nd and 3rd decades of life in the temporal areas of the brain. Grade 3 lesions have a poor prognosis with 40-50% 5-year survival.

Methodology: Here we present the case of a woman with ganglioglioma who presented with a high-grade neuroepithelial tumor thought to represent anaplastic pleomorphic xanthoastrocytoma.

Results: The patient initially presented at 25 years of age with new-onset seizures. Magnetic resonance imaging conducted at the time identified the right anterior temporal mass. She was placed on seizure medication and the mass was completely resected. Pathology revealing WHO grade 1 ganglioglioma. She continued antiseizure medication with two failed attempts to wean off medical treatment. 8 years following her initial resection surveillance MRI showed a new growth in the right temporal lobe. She underwent craniotomy and resection with pathology revealing high-grade neuroepithelial tumor with BRAF V600E mutation initially thought to represent gangliogliosarcoma WHO Grade IV but after a second review with John Hopkins University, was thought to represent WHO Grade III neuroepithelial tumor more suggestive of anaplastic pleomorphic xanthoastrocytoma. MGMT status was negative and IDH-1 and IDH-2 mutations were found to be absent, Ki-67 was initially 32.8%. She was treated postoperatively 60 Gy in 30 fractions following EORTC guidelines for glioblastoma with adjuvant dabrafenib and trametinib. 5 months later she developed right-sided hearing loss attributed to a CSF leak and presented to the OR for craniotomy and correction where intraoperatively tumor regression was noted and resected with pathology confirming high-grade neuro epithelial lesion with BRAF V600E and BCOR mutations and new mutation in NF-1 with elevated Ki-67 to 46%. She was treated adjuvantly with 35 Gy in 10 fractions concomitant temozolomide 75 mg per m2. Unfortunately, the patient continued to have progression following treatment, passing away after one year.

Conclusions: High-grade pleomorphic xanthoastrocytoma is a primary glial tumor with a poor prognosis. It often manifests from transformation from low-grade disease which can bear a close histological resemblance to ganglioglioma. Here treatment was attempted with TKI and chemoradiation following the regimen published by Stupp et al. with limited impact on prognosis and increased aggressive transformation of tumor. Additional research into treatment regimens needs to be conducted to more effectively treat these rare primary glial neoplasms.

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