Pleomorphic xanthoastrocytoma

Giannini, Caterina; Scheithauer, Bernd W.; Burger, Peter C.; Brat, Daniel J.; Wollan, Peter C.; Lach, B.; O’Neill, Brian Patrick

doi:10.1002/(sici)1097-0142(19990501)85:9<2033::aid-cncr22>3.3.co;2-q

Cited by 78 publications

(4 citation statements)

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“…PXA WHO grade II is a slow-growing astrocytic tumor, which is considered benign and presents a 10-years survival rate of 70% and a recurrence-free lapse of 61% [8,33]. However, up to 20% PXA will develop anaplastic features and may further progress to secondary glioblastoma, exhibiting much more aggressive phenotype and dropping significantly survival rates with a median survival time of 15 months [8]. …”

Section: Discussionmentioning

confidence: 99%

“…The biological behavior is usually benign with a 10-years survival rate of 70% and a recurrence-free lapse of 61% [8]. Nevertheless, gradeII PXA may undergo malignant transformation in up to 15-20% [8].…”

Section: Introductionmentioning

confidence: 99%

“…Nevertheless, gradeII PXA may undergo malignant transformation in up to 15-20% [8]. Thus, PXA with elevated mitotic activity (≥ 5 mitoses per 10 high-power fields) and/ or presence of necrosis has been classified as “PXA with anaplastic features” [1,8]. In these cases, the rate of recurrence has been observed to be much higher and the survival time clearly shorter [8].…”

Section: Introductionmentioning

confidence: 99%

“…Thus, PXA with elevated mitotic activity (≥ 5 mitoses per 10 high-power fields) and/ or presence of necrosis has been classified as “PXA with anaplastic features” [1,8]. In these cases, the rate of recurrence has been observed to be much higher and the survival time clearly shorter [8]. These malignant forms are largely responsible for the mortality rate from the disease at 10 years.…”

Section: Introductionmentioning

confidence: 99%

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DNA methylation alterations in grade II- and anaplastic pleomorphic xanthoastrocytoma

et al. 2014

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BackgroundPleomorphic xanthoastrocytoma (PXA) is a rare WHO grade II tumor accounting for less than 1% of all astrocytomas. Malignant transformation into PXA with anaplastic features, is unusual and correlates with poorer outcome of the patients.MethodsUsing a DNA methylation custom array, we have quantified the DNA methylation level on the promoter sequence of 807 cancer-related genes of WHO grade II (n = 11) and III PXA (n = 2) and compared to normal brain tissue (n = 10) and glioblastoma (n = 87) samples. DNA methylation levels were further confirmed on independent samples by pyrosequencing of the promoter sequences.ResultsIncreasing DNA promoter hypermethylation events were observed in anaplastic PXA as compared with grade II samples. We further validated differential hypermethylation of CD81, HCK, HOXA5, ASCL2 and TES on anaplastic PXA and grade II tumors. Moreover, these epigenetic alterations overlap those described in glioblastoma patients, suggesting common mechanisms of tumorigenesis.ConclusionsEven taking into consideration the small size of our patient populations, our data strongly suggest that epigenome-wide profiling of PXA is a valuable tool to identify methylated genes, which may play a role in the malignant progression of PXA. These methylation alterations may provide useful biomarkers for decision-making in those patients with low-grade PXA displaying a high risk of malignant transformation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%