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. 2019 Nov 1;139(2):287–303. doi: 10.1007/s00401-019-02088-8

Table 2.

22 Published pediatric cases of PAAF, with or without DNA methylation profiling

Authors Institutional histopathological diagnosis DNA methylation profile Location Age at diagnosis (years) Gender [M: male; F: female] Death OS (months) Progression PFS (months) Mitotic count/10HPFs Necrosis MAPK alteration Homozygous CDKN2A deletion Loss of ATRX PIK3/ATK alteration Other alterations
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Posterior fossa 0–18 F NA NA NA NA NA NA KIAA1549: BRAF 1 1 NA 0
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Spinal 0–18 F NA NA NA NA NA NA FGFR1 mut/fus 1 0 NA 0
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Posterior fossa 0–18 M NA NA NA NA NA NA FGFR1 mut/fus 0 0 NA MGMT promoter methylation
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Posterior fossa 0–18 M NA NA NA NA NA NA NOS 1 NA NA 0
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Posterior fossa 0–18 M NA NA NA NA NA NA NOS 0 0 NA 0
Reinhardt 2018 Anaplastic pilocytic astrocytoma MC AAP Supratentorial 0–18 F NA NA NA NA NA NA NOS 0 NA NA 0
Lopéz 2018 Anaplastic trans-formation of PA NA Cerebellum 2 F NA NA 1 10 8 0 NTRK2 1 0 0 0
Rodriguez 2011 case 1 /2019 case 17 Anaplastic pilocytic astrocytoma NA Cerebellum 5 M A NA NA NA  ≥ 4 0 BRAF duplication 0 0 pAKT/pS6 IHC
Rodriguez 2011 case 4/2019 case 25 Anaplastic pilocytic astrocytoma NA R frontal lobe 11 M A NA NA NA  ≥ 4 1 NEC 0 0 pAKT/pS6 IHC
Rodriguez 2011 case 10/2019 case 22 Anaplastic pilocytic astrocytoma NA 3rd ventricle 10 M D NA NA NA  ≥ 4 1 NEC 0 1 pAKT/pS6 IHC H3K27M
Rodriguez 2011 case 18 Anaplastic pilocytic astrocytoma NA Spinal 16 M NA NA NA NA  ≥ 4 1 NEC NA NA PTEN het del + pAKT IHC
Rodriguez 2011 case 24/2019 case 26 Anaplastic pilocytic astrocytoma NA R occipital lobe 11 F A NA NA NA  ≥ 4 0 NF1 NA 0 pS6 IHC
Rodriguez 2011 case 25/2019 case 27 Anaplastic pilocytic astrocytoma NA Posterior fossa 14 M D NA NA NA  ≥ 4 1 NOS NA 0 NA
Rodriguez 2019 case 1 PA with anaplasia NA Cerebellum 17 M D NA NA NA  ≥ 5 1 NEC NA 1 0
Rodriguez 2019 case 3 PA with anaplasia NA Cerebellum 13 F A NA NA NA  ≥ 5 1 NOS NA 1 0
Rodriguez 2019 case 4 PA with anaplasia NA Cerebellum 12 F D NA NA NA  ≥ 5 1 NF1 NA 0 0
Rodriguez 2019 case 11 PA with anaplasia NA 3rd ventricle 12 M A NA NA NA  ≥ 5 1 NEC NA 0 0
Rodriguez 2019 case 12 PA with anaplasia NA L hemisphere 3 M A NA NA NA  ≥ 5 1 NEC NA 0 0
Rodriguez 2019 case 13 PA with anaplasia NA Posterior fossa 4 M D NA NA NA  ≥ 5 1 KRAS 0 1 0 H3K27M
Olar 2019 PAAF NA Cerebellum 1.6 M A 49 0 0  ≥ 4 1 KIAA1549: BRAF NA 1 loss of PTEN/ pS6 + IHC
Olar 2019 PAAF NA Cerebellum, midline 12 F A 47 0 0  ≥ 4 1 NOS NA 1 loss of PTEN/ pS6 + IHC
Mistry 2015 PA undergoing trans-formation NA Midline  > 5 F NA NA NA NA NA NA BRAF V600E 1 NA NA

A alive, D deceased, het del heterozygous deletion, IHC immunohistochemistry, MC AAP methylation class anaplastic astrocytoma with piloid features, NA not available, NEC not elsewhere classified, NOS not otherwise specified, PA pilocytic astrocytoma