Anaplastic Pleomorphic Xanthoastrocytomas

Tekkök, İsmail H.; Sav, Aydın

doi:10.1159/000081935

Cited by 25 publications

(8 citation statements)

References 77 publications

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“…Conflicting reports suggest that PXA-AF is more likely to occur primarily or to recur mostly in adults (13, 46, 58, 64). Among the 23 patients with PXA-AF at first diagnosis in our series, adult patients were overrepresented in relation to pediatric patients (16 vs. 7), but neither recurrence-free nor overall survival significantly differed between these age groups.…”

Section: Discussionmentioning

confidence: 99%

“…In the last 15 years, the rare nature of PXA has resulted in a slowly growing literature restricted to well-documented case reports of classic examples (35, 63), classic examples with unusual clinical presentation/course (2, 8, 12, 21, 40, 48, 67) or in uncommon locations (3, 11, 19, 27, 45, 55, 60, 72), rare morphological variants (9, 18, 52, 54, 59, 65, 70), biphasic combined/collision tumors (1, 6, 15, 16, 25, 28, 31, 51, 61, 73), as well as a few small series (17, 20, 29, 38, 39, 42, 47, 53, 58, 62) and reviews (32, 42, 64, 68). Currently, WHO grading of so-called “PXA-AF” remains undefined, and it is still unclear whether these rare tumors should be termed “anaplastic” (WHO grade III).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2014

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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 ≥ 5/10HPF 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/ 10HPF 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 ≥ 5/10HPF. 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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2014

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“…Most of our cases were de novo PXA-As. PXA-A may occur de novo with anaplastic features at first resection or may evolve from PXA grade II to PXA-A, but both have variable, but often poor, outcomes [3, 14-16]. One of our cases had been biopsied elsewhere and diagnosed as anaplastic astrocytoma; sampling error cannot be excluded since the high grade glioma portion of PXA-As is usually indistinguishable from ordinary diffuse high grade glioma, a point made many years ago by Kepes et al .…”

Section: Discussionmentioning

confidence: 99%

Anaplastic PXA in adults: case series with clinicopathologic and molecular features

Schmidt

Kleinschmidt-DeMasters

Aisner

et al. 2012

J Neurooncol

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Background Pleomorphic xanthoastrocytomas with anaplastic features (PXA-As) are rare tumors about which little is known regarding clinicopathological and molecular features. Several studies have identified BRAF V600E mutations in PXA-As, but the percentage with mutation may differ between adult and pediatric examples, and limited information exists about immunohistochemistry for IDH1 (isocitrate dehydrogenase 1). Design: 10 cases of adult PXA-As seen at our institution since 2000 were assessed for BRAF V600E mutation by polymerase chain reaction testing (PCR) and IDH1 by immunohistochemistry. Results Patients ranged in age from 18-68 years; 4 PXA-As affected temporal lobe and 2 were cystic. Four patients underwent gross total resection; and 9/10 received cranial irradiation and/or adjuvant chemotherapy. Five survived less than 5 years, although 2/5 died from non-tumor causes. Four long-term survivors are alive at 7.5, 9.8, 11.4, and 11.9 years post-diagnosis. 2 of 4 long term survivors had BRAF V600E mutation: patients were ages 18 and 28 years. A 48-year old male without BRAF mutation survives at 9.8 years, even with thalamic location; conversely a 68-year-old female with temporal lobe tumor and BRAF mutation survived 1.9 years after diagnosis. All tumors were IDH1 immunonegative. Conclusion This case series details clinicopathological features of a subset of rare PXA-As in adults. BRAF V600E mutation was identified in 50% of these cases.

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“…Both PXAs and PXA-As are more common in children and young adults [7, 9, 10], but well-documented cases have been seen in patients 40 years or older [11, 12, 13, 14, 15, 16]. PXA-A may arise de novo [17, 18], or may develop anaplastic features following recurrence of a previous WHO grade II PXA [12, 14, 17, 18, 19, 20, 21, 22, 23]. Recent reviews by Tekkök et al .…”

Section: Introductionmentioning

confidence: 99%

Epithelioid GBMs Show a High Percentage of BRAF V600E Mutation

Kleinschmidt‐DeMasters

Aisner

Birks

et al. 2013

American Journal of Surgical Pathology

236

214

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Background BRAF V600E mutation has been identified in up to 2/3 of pleomorphic xanthoastrocytomas (PXA), WHO grade II, as well as varying percentages of pleomorphic xanthoastrocytomas with anaplastic features (PXA-A), gangliogliomas, extra-cerebellar pilocytic astrocytomas, and rarely, giant cell GBMs (GC-GBMs). GC-GBMs and epithelioid GBMs (E-GBMs) can be histologically challenging to distinguish from PXA-A. We undertook this study specifically to address whether these 2 tumor types also showed the mutation. Design We tested our originally-reported cohort of 8 E-GBMs and 2 rhabdoid GBMs (Am J Surg Pathol 2010;34:341–354) as well as 5 new E-GBMs (1 pediatric, 4 adult) and 9 GC-GBMs (2 pediatric, 7 adult) (n=24) for BRAF V600E mutational status. 21/24 had sufficient material for IDH1 immunostaining, which is usually absent in PXAs, PXA-As, and primary GBMs, but present in secondary GBMs. Results Patients ranged in age from 4–67 years. BRAF V600E mutation was identified in 7/13 of E-GBMs, including 3 of our original cases; patients with mutation were ages 1050 years. None of the 9 GC-GBMs or 2 rhabdoid GBMs manifested this mutation, including pediatric patients. The sole secondary E-GBM was the single case manifesting positive IDH1 immunoreactivity. Conclusion A high percentage of E-GBMs manifest BRAF V600E mutation, paralleling PXAs. All rhabdoid GBMs and GC-GBMs were negative, although larger multi-institutional cohorts will have to be tested to extend this result. BRAF V600E mutational analyses should be performed on E-GBMs, particularly in all pediatric and young-aged adults, given the potential for BRAF inhibitor therapy in this subset of GBM patients.

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Anaplastic Pleomorphic Xanthoastrocytomas

Cited by 25 publications

References 77 publications

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

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

Anaplastic PXA in adults: case series with clinicopathologic and molecular features

Epithelioid GBMs Show a High Percentage of BRAF V600E Mutation

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