Pleomorphic Xanthoastrocytoma with Anaplastic Features: Retrospective Case Series

Rutkowski, Martin J.; Oh, Taemin; Gulniflioglu, Gulen; Safaee, Michael; Tihan, Tarık; Parsa, Andrew T.

doi:10.1016/j.wneu.2016.07.068

Cited by 27 publications

(18 citation statements)

References 45 publications

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“…In contrast, only 2 PXA cases showed heterogeneous enhancement, and 3 cases had marked peritumoral edema. The enhancement pattern of APXA could be explained by the presence of necrosis, hemorrhage, and vascular proliferation within the tumor, which was observed in most APXAs by Rutkowski et al 11 Additionally, our cohort showed results similar to those in a previous study for the frequency of the peritumoral edema. 20 In the study of Lim et al, 20 all PXAs with anaplastic features (4 cases) had severe perilesional edema, while only 1 of 18 PXAs presented with severe peritumoral edema, 6 cases had none, 8 cases had mild edema, and 3 cases had moderate edema.…”

Section: Discussionsupporting

confidence: 88%

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MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma

She¹,

Liu²,

Zhou³

et al. 2018

AJNR Am J Neuroradiol

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Section: Discussionsupporting

confidence: 88%

“…5,10 Furthermore, APXA has been reported to have a worse prognosis than PXA, with 5-year overall survival of 57.1%. 5,11 Therefore, preoperative differentiation between the 2 entities by MR imaging may aid in planning the treatment strategy and predicting prognosis.…”

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confidence: 99%

MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma

She¹,

Liu²,

Zhou³

et al. 2018

AJNR Am J Neuroradiol

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“…eGBM and PXA exhibit some common findings on MRI: (i) contrast‐enhanced solid and/or cystic; (ii) well circumscribed with no or little peritumoral edema; (iii) leptomeningeal involvement; and (iv) leptomeningeal dissemination compared with diffuse glioma at recurrence . In our case series, one eGBM (case 15, Figs ) totally excised at first operation has been alive for over 2 years.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological and genetic association between epithelioid glioblastoma and pleomorphic xanthoastrocytoma

et al. 2018

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Epithelioid glioblastoma (eGBM) is a rare variant of GBM which was adopted in the 2016 WHO classification. eGBM and pleomorphic xanthoastrocytoma (PXA) sometimes show overlapping features histologically and genetically, such as epithelioid pattern and a highly frequent V600E mutation in the gene for vRAF murine sarcoma viral oncogene homolog B1 (BRAF), respectively. Accurate diagnosis of these rare tumors is challenging according to the new criteria in the revised 2016 WHO classification. It is an urgent task to elucidate the biological properties of the tumors and to select appropriate treatment. Twenty consecutive cases diagnosed as PXA or eGBM histologically were investigated. Twelve of the 20 cases were PXAs and eight were eGBMs. Morphologically, mitotic activity, necrosis and degenerative changes such as intracellular lipid accumulation, eosinophilic granular bodies and reticulin fiber deposits were scored. Immunohistochemical and molecular biological assessment for isocitrate dehydrogenases 1 and 2 (IDH1/2), α-thalassemia/mental-retardation-syndrome-X-linked gene (ATRX), p53, BRAF, telomere reverse transcriptase promoter (TERT-p), H3F3A, and integrase interactor 1 (INI1) were performed. eGBM tended to lack the degenerative changes characteristic for PXA. Of the 20 cases tested, Sanger technique showed no mutation in IDH1/2. BRAF mutation at T1799 > A (V600E) was detected in 4/12 (33.3%) PXA and 4/8 (50.0%) eGBM, while TERT-p mutation was detected at C228 > T in 2/12 (16.7%) PXA and at C250 > T in 1/8 (12.5%) eGBM. Retained nuclear ATRX was observed in 12/12 (100%) PXA and 6/7 (85.7%) eGBM while p53 mutation was observed in 2/10 (20%) PXA and 7/7 (100%) eGBM. All tumors retained INI1 expression in their nuclei. None of the tumors harbored H3F3A mutation. One PXA without BRAF mutation acquired TERT-p mutation at recurrence and one eGBM harbored both BRAF and TERT-p mutation. Molecular biological similarity between eGBM and PXA was suggested in our series, while degenerative changes reflected the features of PXA. It was speculated that the common genetic alterations for development and progression of eGBM and PXA might include BRAF and TERT-p mutations.

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“…The 3‐year progression‐free survival (PFS) was 32% for aPXAs compared with 80% for grade II PXAs. Another retrospective series of eight patients with aPXA demonstrated a 1‐year PFS of 57% . In this cohort, six patients had subtotal resections and all progressed despite adjuvant radiation and chemotherapy.…”

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confidence: 76%

Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab

Thomas

Tucker

Nelson

et al. 2018

Pediatric Blood & Cancer

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Our experience suggests that targeted therapy with dabrafenib and trametinib can be safely combined with antiangiogenic therapy and may improve quality of life and survival in patients with leptomeningeal disease associated with aPXA. To our knowledge, this is the first time dabrafenib, trametinib, and bevacizumab have been combined to treat a pediatric high-grade glioma.Anaplastic PXAs pose a therapeutic challenge because of their rarity and lack of consensus guidelines for treatment. The natural history of these tumors is poorly described, but a recent review of 37 patients with PXA reclassified by the 2016 WHO classification and found that 20% fulfilled criteria for aPXA. 7 All patients were treated with maximal safe resection and those with a MIB-1 of greater than 5% or with residual disease were treated with adjuvant RT. The 3-year progression-free survival (PFS) was 32% for aPXAs compared with 80% for grade II PXAs. Another retrospective series of eight patients with aPXA demonstrated a 1-year PFS of 57%. 8 In this cohort, six patients had subtotal resections and all progressed despite adjuvant radiation and chemotherapy. Among them, four patients developed disseminated disease. Few treatments have been shown to be effective in leptomeningeal carcinomatosis from gliomas, but bevacizumab can improve symptom control through its antiangiogenic effects and normalization of the blood-brain barrier. 9Mutations in the oncogene BRAF have been reported in 40-60% of PXAs. 2 A basket study of vemurafenib for nonmelanoma cancers with BRAF V600E mutations included four patients with recurrent aPXAs, three of whom had a partial response. 3 Our case report and others 4,10 suggest that BRAF-mutant aPXAs may be sensitive to targeted therapy, even if disseminated or refractory to other treatments. The growing experience with targeted therapy in rare pediatric gliomas justifies a need for a larger clinical trial. Multicenter national clinical trials for recurrent/refractory BRAF-mutant pediatric glioma are ongoing (NCT01748149) and will lead to better understanding of how to manage these challenging tumors. CONFLICT OF INTERESTThe authors declare that there is no conflict of interest. ORCID Alissa A. Thomas

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Pleomorphic Xanthoastrocytoma with Anaplastic Features: Retrospective Case Series

Cited by 27 publications

References 45 publications

MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma

MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma

Clinicopathological and genetic association between epithelioid glioblastoma and pleomorphic xanthoastrocytoma

Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab

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