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. 2015 Sep;25(5):575-86.
doi: 10.1111/bpa.12217. Epub 2014 Dec 5.

Pleomorphic Xanthoastrocytoma: Natural History and Long-Term Follow-Up

Cristiane M Ida  1 , Fausto J Rodriguez  2 , Peter C Burger  2 , Alissa A Caron  1 , Sarah M Jenkins  3 , Grant M Spears  3 , Dawn L Aranguren  4 , Daniel H Lachance  4 , Caterina Giannini  1
Affiliations

Pleomorphic Xanthoastrocytoma: Natural History and Long-Term Follow-Up

Cristiane M Ida et al. Brain Pathol. 2015 Sep.

Abstract

Prognostic significance of histological anaplasia and BRAF V600E mutation were retrospectively evaluated in 74 patients with pleomorphic xanthoastrocytoma (PXA). Median age at diagnosis was 21.5 years (31 pediatric, 43 adult) and median follow-up 7.6 years. Anaplasia (PXA-AF), defined as mitotic index ≥ 5/10 HPF and/or presence of necrosis, was present in 33 cases. BRAF V600E mutation was detected in 39 (of 60) cases by immunohistochemical and/or molecular analysis, all negative for IDH1 (R132H). Mitotic index ≥ 5/10 HPF and necrosis were associated with decreased overall survival (OS; P = 0.0005 and P = 0.0002, respectively). In all cases except two, necrosis was associated with mitotic index ≥ 5/10 HPF. Patients with BRAF V600E mutant tumors had significantly longer OS compared with those without BRAF V600E mutation (P = 0.02). PXA-AF patients, regardless of age, had significantly shorter OS compared with those without (P = 0.0003). Recurrence-free survival was significantly shorter for adult PXA-AF patients (P = 0.047) only. Patients who either recurred or died ≤ 3 years from diagnosis were more likely to have had either PXA-AF at first diagnosis (P = 0.008) or undergone a non-gross total resection procedure (P = 0.004) as compared with patients who did not. This study provides further evidence that PXA-AF behaves more aggressively than PXA and may qualify for WHO grade III "anaplastic" designation.

Keywords: BRAF V600E; IDH1 R132H; WHO grade; anaplastic; glioma; pleomorphic xanthoastrocytoma.

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Figures

Figure 1
Figure 1
A,B. Characteristic histological features of PXA. A. Leptomeningeal involvement by pleomorphic, giant and spindle‐shaped tumor cells. H&E, 100×. B. Pale and intensively eosinophilic granular bodies with occasional xanthic tumor cells. H&E, 400×. C–E. Histological features of anaplasia. C. Mitotic index ≥ 5/10 HPF, including atypical mitotic figures. H&E. 400×. D. Necrosis and E. endothelial proliferation. H&E, 200×. F. BRAF V600E immunostain: strong labeling of tumor cells. 200×.
Figure 2
Figure 2
A,B. Recurrence‐free and overall survival for the entire PXA group. C,D. Recurrence‐free and overall survival stratified by diagnosis at first resection: recurrence‐free survival was slightly longer for patients with PXA when compared with patients with PXA‐AF (P = 0.09). PXA‐AF patients showed a significant decreased overall survival when compared with patients with PXA (P = 0.0003).
Figure 3
Figure 3
A,B. Recurrence‐free and overall survival by age group: similar recurrence‐free and overall survival for PXA pediatric vs. adult patients.
Figure 4
Figure 4
A,B. Recurrence‐free and overall survival stratified by diagnosis at first resection in the pediatric group: although recurrence‐free survival was similar between classic PXA and PXA‐AF patients, overall survival of patients with PXA‐AF was significantly shorter as compared with patients with PXA. C,D. Recurrence‐free and overall survival stratified by diagnosis at first resection in the adult group: PXA‐AF patients showed significantly decreased recurrence‐free and overall survival when compared with adult patients with PXA.
Figure 5
Figure 5
Overall survival for the entire PXA group stratified by (A) mitotic index (MI) and (B) tumor necrosis: MI ≥ 5/10HPF and presence of tumor necrosis are significantly associated with decreased overall survival.
Figure 6
Figure 6
Overall survival for the entire PXA group stratified by BRAF V600E mutation status: patients with BRAF V600E mutant tumors had significantly longer overall survival when compared with those with BRAF V600E nonmutant tumors (P = 0.02). Adjusted multivariate analyses because of the higher frequency of BRAF V600E mutation among PXA patients in comparison with PXA‐AF patients could not be performed.
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