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Anaplastic pleomorphic xanthoastrocytoma

MedGen UID:
924393
•Concept ID:
C4283858
•
Neoplastic Process
Synonyms: Anaplastic Pleomorphic Xanthoastrocytoma; anaplastic pleomorphic xanthoastrocytoma; WHO Grade 3 Pleomorphic Xanthoastrocytoma; WHO Grade III Pleomorphic Xanthoastrocytoma
SNOMED CT: Anaplastic pleomorphic xanthoastrocytoma (733848006)
 
Monarch Initiative: MONDO:0850312

Definition

A WHO grade 3 pleomorphic xanthoastrocytoma characterized by the presence of five or more mitoses per 10 high-power fields. Necrosis may be present. Patients have shorter survival rates when compared to those with WHO grade 2 pleomorphic xanthoastrocytoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnaplastic pleomorphic xanthoastrocytoma

Professional guidelines

PubMed

Pediatric Pleomorphic Xanthoastrocytoma: A National Database Inquiry on Current Treatment Approaches in the United States.
Scarpelli DB, Yu Y, Tep AC, Bergue B, Degnin C, Chen Y, McClelland S 3rd, Jaboin JJ
Cancer Rep (Hoboken) 2021 Dec;4(6):e1415. Epub 2021 May 8 doi: 10.1002/cnr2.1415. PMID: 33963808Free PMC Article

Recent clinical studies

Etiology

Identification of BRAF p. V600E-Mutant and Wild-Type by MR Imaging in Pleomorphic Xanthoastrocytoma and Anaplastic Pleomorphic Xanthoastrocytoma.
Huang W, Cai J, Lin N, Xu Y, Wang H, Wu Z, Kang D
AJNR Am J Neuroradiol 2021 Dec;42(12):2152-2159. Epub 2021 Nov 1 doi: 10.3174/ajnr.A7324. PMID: 34725042Free PMC Article
MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma.
She D, Liu J, Xing Z, Zhang Y, Cao D, Zhang Z
AJNR Am J Neuroradiol 2018 Aug;39(8):1446-1452. Epub 2018 Jun 14 doi: 10.3174/ajnr.A5701. PMID: 29903923Free PMC Article
WHO 2016 Classification of gliomas.
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Recurrent copy number alterations in low-grade and anaplastic pleomorphic xanthoastrocytoma with and without BRAF V600E mutation.
Vaubel RA, Caron AA, Yamada S, Decker PA, Eckel Passow JE, Rodriguez FJ, Nageswara Rao AA, Lachance D, Parney I, Jenkins R, Giannini C
Brain Pathol 2018 Mar;28(2):172-182. Epub 2017 Apr 2 doi: 10.1111/bpa.12495. PMID: 28181325Free PMC Article
DNA methylation alterations in grade II- and anaplastic pleomorphic xanthoastrocytoma.
Martínez R, Carmona FJ, Vizoso M, Rohde V, Kirsch M, Schackert G, Ropero S, Paulus W, Barrantes A, Gomez A, Esteller M
BMC Cancer 2014 Mar 20;14:213. doi: 10.1186/1471-2407-14-213. PMID: 24650279Free PMC Article

Diagnosis

BRAF/MEK Dual Inhibitors Therapy in Progressive and Anaplastic Pleomorphic Xanthoastrocytoma: Case Series and Literature Review.
Kata K, Rodriguez-Quintero JC, Arevalo OD, Zhang JJ, Bhattacharjee MB, Ware C, Dono A, Riascos-Castaneda R, Tandon N, Blanco A, Esquenazi Y, Ballester LY, Amsbaugh M, Day AL, Zhu JJ
J Natl Compr Canc Netw 2022 Nov;20(11):1193-1202.e6. doi: 10.6004/jnccn.2022.7046. PMID: 36351333
Pleomorphic xanthoastrocytoma, anaplastic pleomorphic xanthoastrocytoma, and epithelioid glioblastoma: Case series with clinical characteristics, molecular features and progression relationship.
Lin Z, Yang R, Zheng H, Li Z, Yi G, Wu Q, Yang C, Huang G
Clin Neurol Neurosurg 2022 Oct;221:107379. Epub 2022 Jul 21 doi: 10.1016/j.clineuro.2022.107379. PMID: 35932588
Anaplastic pleomorphic xanthoastrocytoma associated with an H3G34 mutation: a case report with review of literature.
Sasaki S, Tomomasa R, Nobusawa S, Hirato J, Uchiyama T, Boku E, Miyasaka T, Hirose T, Ohbayashi C
Brain Tumor Pathol 2019 Oct;36(4):169-173. Epub 2019 Jul 26 doi: 10.1007/s10014-019-00349-8. PMID: 31350684
WHO 2016 Classification of gliomas.
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Anaplastic pleomorphic xanthoastrocytoma.
Bayindir C, Balak N, Karasu A, Kasaroğlu D
Childs Nerv Syst 1997 Jan;13(1):50-6. doi: 10.1007/s003810050040. PMID: 9083703

Therapy

BRAF/MEK Dual Inhibitors Therapy in Progressive and Anaplastic Pleomorphic Xanthoastrocytoma: Case Series and Literature Review.
Kata K, Rodriguez-Quintero JC, Arevalo OD, Zhang JJ, Bhattacharjee MB, Ware C, Dono A, Riascos-Castaneda R, Tandon N, Blanco A, Esquenazi Y, Ballester LY, Amsbaugh M, Day AL, Zhu JJ
J Natl Compr Canc Netw 2022 Nov;20(11):1193-1202.e6. doi: 10.6004/jnccn.2022.7046. PMID: 36351333
Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article
Complete Remission of an Extracranially Disseminated Anaplastic Pleomorphic Xanthoastrocytoma With Everolimus: A Case Report and Literature Review.
Saraf AJ, Elhawary G, Finlay JL, Scott S, Olshefski R, Halverson M, Boue DR, AbdelBaki MS
Pediatr Neurol 2018 Nov;88:65-70. Epub 2018 Sep 13 doi: 10.1016/j.pediatrneurol.2018.09.004. PMID: 30322731
Sustained Response to Targeted Therapy in a Patient With Disseminated Anaplastic Pleomorphic Xanthoastrocytoma.
Amayiri N, Swaidan M, Al-Hussaini M, Halalsheh H, Al-Nassan A, Musharbash A, Tabori U, Hawkins C, Bouffet E
J Pediatr Hematol Oncol 2018 Aug;40(6):478-482. doi: 10.1097/MPH.0000000000001032. PMID: 29200156
Dabrafenib in BRAFV600-mutated anaplastic pleomorphic xanthoastrocytoma.
Brown NF, Carter T, Mulholland P
CNS Oncol 2017 Jan;6(1):5-9. Epub 2016 Oct 26 doi: 10.2217/cns-2016-0031. PMID: 27781490Free PMC Article

Prognosis

Identification of BRAF p. V600E-Mutant and Wild-Type by MR Imaging in Pleomorphic Xanthoastrocytoma and Anaplastic Pleomorphic Xanthoastrocytoma.
Huang W, Cai J, Lin N, Xu Y, Wang H, Wu Z, Kang D
AJNR Am J Neuroradiol 2021 Dec;42(12):2152-2159. Epub 2021 Nov 1 doi: 10.3174/ajnr.A7324. PMID: 34725042Free PMC Article
Anaplastic pleomorphic xanthoastrocytoma associated with an H3G34 mutation: a case report with review of literature.
Sasaki S, Tomomasa R, Nobusawa S, Hirato J, Uchiyama T, Boku E, Miyasaka T, Hirose T, Ohbayashi C
Brain Tumor Pathol 2019 Oct;36(4):169-173. Epub 2019 Jul 26 doi: 10.1007/s10014-019-00349-8. PMID: 31350684
The genetic landscape of anaplastic pleomorphic xanthoastrocytoma.
Phillips JJ, Gong H, Chen K, Joseph NM, van Ziffle J, Bastian BC, Grenert JP, Kline CN, Mueller S, Banerjee A, Nicolaides T, Gupta N, Berger MS, Lee HS, Pekmezci M, Tihan T, Bollen AW, Perry A, Shieh JTC, Solomon DA
Brain Pathol 2019 Jan;29(1):85-96. Epub 2018 Nov 6 doi: 10.1111/bpa.12639. PMID: 30051528Free PMC Article
MR Imaging Features of Anaplastic Pleomorphic Xanthoastrocytoma Mimicking High-Grade Astrocytoma.
She D, Liu J, Xing Z, Zhang Y, Cao D, Zhang Z
AJNR Am J Neuroradiol 2018 Aug;39(8):1446-1452. Epub 2018 Jun 14 doi: 10.3174/ajnr.A5701. PMID: 29903923Free PMC Article
Anaplastic pleomorphic xanthoastrocytoma.
Bayindir C, Balak N, Karasu A, Kasaroğlu D
Childs Nerv Syst 1997 Jan;13(1):50-6. doi: 10.1007/s003810050040. PMID: 9083703

Clinical prediction guides

Identification of BRAF p. V600E-Mutant and Wild-Type by MR Imaging in Pleomorphic Xanthoastrocytoma and Anaplastic Pleomorphic Xanthoastrocytoma.
Huang W, Cai J, Lin N, Xu Y, Wang H, Wu Z, Kang D
AJNR Am J Neuroradiol 2021 Dec;42(12):2152-2159. Epub 2021 Nov 1 doi: 10.3174/ajnr.A7324. PMID: 34725042Free PMC Article
BRAF V600E, TERT, and IDH2 Mutations in Pleomorphic Xanthoastrocytoma: Observations from a Large Case-Series Study.
Ma C, Feng R, Chen H, Hameed NUF, Aibaidula A, Song Y, Wu J
World Neurosurg 2018 Dec;120:e1225-e1233. Epub 2018 Sep 18 doi: 10.1016/j.wneu.2018.09.050. PMID: 30240866
DNA methylation alterations in grade II- and anaplastic pleomorphic xanthoastrocytoma.
Martínez R, Carmona FJ, Vizoso M, Rohde V, Kirsch M, Schackert G, Ropero S, Paulus W, Barrantes A, Gomez A, Esteller M
BMC Cancer 2014 Mar 20;14:213. doi: 10.1186/1471-2407-14-213. PMID: 24650279Free PMC Article
Anaplastic pleomorphic xanthoastrocytoma.
Prayson RA, Morris HH 3rd
Arch Pathol Lab Med 1998 Dec;122(12):1082-6. PMID: 9870856
Anaplastic pleomorphic xanthoastrocytoma.
Bayindir C, Balak N, Karasu A, Kasaroğlu D
Childs Nerv Syst 1997 Jan;13(1):50-6. doi: 10.1007/s003810050040. PMID: 9083703

Recent systematic reviews

Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article

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