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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.
 
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.

Professional guidelines

PubMed

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
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
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Recent clinical studies

Etiology

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
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
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007
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
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663

Diagnosis

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
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
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
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
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

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: 37004712
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
Khalafallah AM, Rakovec M, Mukherjee D
Clin Neurol Neurosurg 2020 Sep;196:106042. Epub 2020 Jun 24 doi: 10.1016/j.clineuro.2020.106042. PMID: 32599422
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007
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

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
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
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
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
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

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
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
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007
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
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

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
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
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
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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