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Pleomorphic xanthoastrocytoma(PXA)

MedGen UID:
137786
•Concept ID:
C0334586
•
Neoplastic Process
Synonym: PXA
SNOMED CT: Pleomorphic xanthoastrocytoma (78838008); Pleomorphic xanthoastrocytoma (189924002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0033682
Monarch Initiative: MONDO:0016690
Orphanet: ORPHA251607

Definition

Pleomorphic xanthoastrocytomas (PXA) are rare low-grade astrocytomas (WHO Grade II) typically found in the temporal lobe and classically presenting with epilepsy. PXA is an astrocytic neoplasm that most often presents in children or young adults but can also occur in adults. The diagnosis of anaplastic PXA is made based upon tumor histopathologic characteristics and requires increased proliferative activity (mitotic index at least 5 mitoses/10 HPF), which is associated with worse overall survival. In general, anaplastic PXAs acquire features of a more aggressive astrocytic neoplasm that can include increased proliferation, necrosis, microvascular proliferation, loss of pericellular reticulin, and increased infiltrative growth. [from HPO]

Conditions with this feature

Mismatch repair cancer syndrome 1
MedGen UID:
1748029
•Concept ID:
C5399763
•
Disease or Syndrome
Lynch syndrome is characterized by an increased risk for colorectal cancer (CRC) and cancers of the endometrium, ovary, stomach, small bowel, urinary tract, biliary tract, brain (usually glioblastoma), skin (sebaceous adenomas, sebaceous carcinomas, and keratoacanthomas), pancreas, and prostate. Cancer risks and age of onset vary depending on the associated gene. Several other cancer types have been reported to occur in individuals with Lynch syndrome (e.g., breast, sarcomas, adrenocortical carcinoma). However, the data are not sufficient to demonstrate that the risk of developing these cancers is increased in individuals with Lynch syndrome.
See: Condition Record
Mismatch repair cancer syndrome 1

Professional guidelines

PubMed

pRB immunostaining in the differential diagnosis between pleomorphic xanthoastrocytoma and glioblastoma with giant cells.
Barresi V, Simbolo M, Ciaparrone C, Pedron S, Mafficini A, Scarpa A
Histopathology 2022 Nov;81(5):661-669. Epub 2022 Aug 25 doi: 10.1111/his.14768. PMID: 35945679Free PMC Article
Integrated genotype-phenotype analysis of long-term epilepsy-associated ganglioglioma.
Wang Y, Wang L, Blümcke I, Zhang W, Fu Y, Shan Y, Piao Y, Zhao G
Brain Pathol 2022 Jan;32(1):e13011. Epub 2021 Aug 5 doi: 10.1111/bpa.13011. PMID: 34355449Free PMC Article
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Recent clinical studies

Etiology

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Pleomorphic Xanthoastrocytoma: a single institution retrospective analysis and a review of the literature.
Detti B, Scoccianti S, Maragna V, Lucidi S, Ganovelli M, Teriaca MA, Caini S, Desideri I, Agresti B, Greto D, Buccoliero AM, Puppa AD, Sardi I, Livi L
Radiol Med 2022 Oct;127(10):1134-1141. Epub 2022 Aug 11 doi: 10.1007/s11547-022-01531-3. PMID: 35951279Free PMC Article
Impact of Grade on Survival in Pleomorphic Xanthoastrocytoma and Low Prevalence of BRAF V600E Mutation.
Gandham EJ, Goyal-Honavar A, Beno D, Pai R, Balakrishan R, Jasper A, Gowri M, Moorthy RK, Chacko AG, Chacko G
World Neurosurg 2022 Aug;164:e922-e928. Epub 2022 May 23 doi: 10.1016/j.wneu.2022.05.066. PMID: 35618235
Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.
Stichel D, Ebrahimi A, Reuss D, Schrimpf D, Ono T, Shirahata M, Reifenberger G, Weller M, Hänggi D, Wick W, Herold-Mende C, Westphal M, Brandner S, Pfister SM, Capper D, Sahm F, von Deimling A
Acta Neuropathol 2018 Nov;136(5):793-803. Epub 2018 Sep 5 doi: 10.1007/s00401-018-1905-0. PMID: 30187121
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

Diagnosis

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
High-grade glioma with pleomorphic and pseudopapillary features (HPAP): a proposed type of circumscribed glioma in adults harboring frequent TP53 mutations and recurrent monosomy 13.
Pratt D, Abdullaev Z, Papanicolau-Sengos A, Ketchum C, Panneer Selvam P, Chung HJ, Lee I, Raffeld M, Gilbert MR, Armstrong TS, Pytel P, Borys E, Klonoski JM, McCord M, Horbinski C, Brat D, Perry A, Solomon D, Eberhart C, Giannini C, Quezado M, Aldape K
Acta Neuropathol 2022 Mar;143(3):403-414. Epub 2022 Feb 1 doi: 10.1007/s00401-022-02404-9. PMID: 35103816Free PMC Article
Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.
Stichel D, Ebrahimi A, Reuss D, Schrimpf D, Ono T, Shirahata M, Reifenberger G, Weller M, Hänggi D, Wick W, Herold-Mende C, Westphal M, Brandner S, Pfister SM, Capper D, Sahm F, von Deimling A
Acta Neuropathol 2018 Nov;136(5):793-803. Epub 2018 Sep 5 doi: 10.1007/s00401-018-1905-0. PMID: 30187121
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
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368

Therapy

Response Rate and Molecular Correlates to Encorafenib and Binimetinib in BRAF-V600E Mutant High-Grade Glioma.
Schreck KC, Strowd RE, Nabors LB, Ellingson BM, Chang M, Tan SK, Abdullaev Z, Turakulov R, Aldape K, Danda N, Desideri S, Fisher J, Iacoboni M, Surakus T, Rudek MA, Bettegowda C, Grossman SA, Ye X
Clin Cancer Res 2024 May 15;30(10):2048-2056. doi: 10.1158/1078-0432.CCR-23-3241. PMID: 38446982Free PMC Article
Recombinant polio-rhinovirus immunotherapy for recurrent paediatric high-grade glioma: a phase 1b trial.
Thompson EM, Landi D, Brown MC, Friedman HS, McLendon R, Herndon JE 2nd, Buckley E, Bolognesi DP, Lipp E, Schroeder K, Becher OJ, Friedman AH, McKay Z, Walter A, Threatt S, Jaggers D, Desjardins A, Gromeier M, Bigner DD, Ashley DM
Lancet Child Adolesc Health 2023 Jul;7(7):471-478. Epub 2023 Mar 30 doi: 10.1016/S2352-4642(23)00031-7. PMID: 37004712Free PMC Article
Sporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors.
Kim H, Lim KY, Park JW, Kang J, Won JK, Lee K, Shim Y, Park CK, Kim SK, Choi SH, Kim TM, Yun H, Park SH
Lab Invest 2022 Feb;102(2):160-171. Epub 2021 Nov 30 doi: 10.1038/s41374-021-00694-3. PMID: 34848827Free PMC Article
Evidence-Based Practice: Temozolomide Beyond Glioblastoma.
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article

Prognosis

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
The evolution of pleomorphic xanthoastrocytoma: from genesis to molecular alterations and mimics.
Mahajan S, Dandapath I, Garg A, Sharma MC, Suri V, Sarkar C
Lab Invest 2022 Jul;102(7):670-681. Epub 2022 Jan 14 doi: 10.1038/s41374-021-00708-0. PMID: 35031693
Pleomorphic xanthoastrocytoma: a brief review.
Shaikh N, Brahmbhatt N, Kruser TJ, Kam KL, Appin CL, Wadhwani N, Chandler J, Kumthekar P, Lukas RV
CNS Oncol 2019 Nov 1;8(3):CNS39. Epub 2019 Sep 19 doi: 10.2217/cns-2019-0009. PMID: 31535562Free PMC Article
Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.
Stichel D, Ebrahimi A, Reuss D, Schrimpf D, Ono T, Shirahata M, Reifenberger G, Weller M, Hänggi D, Wick W, Herold-Mende C, Westphal M, Brandner S, Pfister SM, Capper D, Sahm F, von Deimling A
Acta Neuropathol 2018 Nov;136(5):793-803. Epub 2018 Sep 5 doi: 10.1007/s00401-018-1905-0. PMID: 30187121
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368

Clinical prediction guides

Pleomorphic Xanthoastrocytoma: a single institution retrospective analysis and a review of the literature.
Detti B, Scoccianti S, Maragna V, Lucidi S, Ganovelli M, Teriaca MA, Caini S, Desideri I, Agresti B, Greto D, Buccoliero AM, Puppa AD, Sardi I, Livi L
Radiol Med 2022 Oct;127(10):1134-1141. Epub 2022 Aug 11 doi: 10.1007/s11547-022-01531-3. PMID: 35951279Free PMC Article
Pleomorphic xanthoastrocytoma: a brief review.
Shaikh N, Brahmbhatt N, Kruser TJ, Kam KL, Appin CL, Wadhwani N, Chandler J, Kumthekar P, Lukas RV
CNS Oncol 2019 Nov 1;8(3):CNS39. Epub 2019 Sep 19 doi: 10.2217/cns-2019-0009. PMID: 31535562Free PMC Article
Evidence-Based Practice: Temozolomide Beyond Glioblastoma.
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007
Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.
Stichel D, Ebrahimi A, Reuss D, Schrimpf D, Ono T, Shirahata M, Reifenberger G, Weller M, Hänggi D, Wick W, Herold-Mende C, Westphal M, Brandner S, Pfister SM, Capper D, Sahm F, von Deimling A
Acta Neuropathol 2018 Nov;136(5):793-803. Epub 2018 Sep 5 doi: 10.1007/s00401-018-1905-0. PMID: 30187121
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article

Recent systematic reviews

The Significance of BRAF Mutation in the Epithelioid Glioblastoma Subtype: A Systematic Literature Review and a Case Report with a Unique Intraventricular Topography.
Prieto R, Barrios L, Ebrat-Mancilla E, Martín P, Tejerina E
Int J Surg Pathol 2024 Jun;32(4):649-666. Epub 2023 Sep 24 doi: 10.1177/10668969231195026. PMID: 37743598
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
Cerebellar pleomorphic xanthoastrocytoma in the setting of neurofibromatosis type-I: Does it portend a different prognosis? A case report and systematic review.
Mathkour M, Banerjee S, Werner C, Hanna J, Abou-Al-Shaar H, Dindial R, Scullen T, Boehm L, Tubbs RS, Ware ML
Clin Neurol Neurosurg 2021 Jan;200:106346. Epub 2020 Nov 2 doi: 10.1016/j.clineuro.2020.106346. PMID: 33160715
Clinical relevance of BRAF status in glial and glioneuronal tumors: A systematic review.
Sugiura Y, Nagaishi M
J Clin Neurosci 2019 Aug;66:196-201. Epub 2019 May 27 doi: 10.1016/j.jocn.2019.05.014. PMID: 31147232
Grade II Pleomorphic Xanthoastrocytoma; a meta-analysis of data from previously reported 167 cases.
Mallick S, Benson R, Melgandi W, Giridhar P, Rath GK
J Clin Neurosci 2018 Aug;54:57-62. Epub 2018 May 24 doi: 10.1016/j.jocn.2018.05.003. PMID: 29803334

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