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Pilocytic astrocytoma

MedGen UID:
87271
•Concept ID:
C0334583
•
Neoplastic Process
Synonym: Pilocytic astrocytoma, somatic
SNOMED CT: Piloid astrocytoma (128854008); Pilocytic astrocytoma (128854008); Pilocytic astrocytoma (763865009)
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:0033680
Monarch Initiative: MONDO:0016691
Orphanet: ORPHA251612

Definition

The most common form of astrocytoma (WHO Grade I) in childhood. These typically have MAPK signalling pathway abnormalities. [from HPO]

Professional guidelines

PubMed

Adult Intramedullary Pilocytic Astrocytomas: Clinical Features, Management, and Outcomes.
Yang T, Wu H, Xia C
Neurol India 2022 Sep-Oct;70(Supplement):S206-S210. doi: 10.4103/0028-3886.360936. PMID: 36412370
Conventional chemotherapy and perspectives for molecular-based oncological treatment in pediatric hemispheric low-grade gliomas.
Fernández MB, Alonso VP
Childs Nerv Syst 2016 Oct;32(10):1939-45. Epub 2016 Sep 20 doi: 10.1007/s00381-016-3132-0. PMID: 27659836
The Role of Molecular Diagnostics in the Management of Patients with Gliomas.
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915

Recent clinical studies

Etiology

Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free 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
BRAF Inhibition in BRAF(V600)-Mutant Gliomas: Results From the VE-BASKET Study.
Kaley T, Touat M, Subbiah V, Hollebecque A, Rodon J, Lockhart AC, Keedy V, Bielle F, Hofheinz RD, Joly F, Blay JY, Chau I, Puzanov I, Raje NS, Wolf J, DeAngelis LM, Makrutzki M, Riehl T, Pitcher B, Baselga J, Hyman DM
J Clin Oncol 2018 Dec 10;36(35):3477-3484. Epub 2018 Oct 23 doi: 10.1200/JCO.2018.78.9990. PMID: 30351999Free PMC Article
Anaplastic astrocytoma with piloid features, a novel molecular class of IDH wildtype glioma with recurrent MAPK pathway, CDKN2A/B and ATRX alterations.
Reinhardt A, Stichel D, Schrimpf D, Sahm F, Korshunov A, Reuss DE, Koelsche C, Huang K, Wefers AK, Hovestadt V, Sill M, Gramatzki D, Felsberg J, Reifenberger G, Koch A, Thomale UW, Becker A, Hans VH, Prinz M, Staszewski O, Acker T, Dohmen H, Hartmann C, Mueller W, Tuffaha MSA, Paulus W, Heß K, Brokinkel B, Schittenhelm J, Monoranu CM, Kessler AF, Loehr M, Buslei R, Deckert M, Mawrin C, Kohlhof P, Hewer E, Olar A, Rodriguez FJ, Giannini C, NageswaraRao AA, Tabori U, Nunes NM, Weller M, Pohl U, Jaunmuktane Z, Brandner S, Unterberg A, Hänggi D, Platten M, Pfister SM, Wick W, Herold-Mende C, Jones DTW, von Deimling A, Capper D
Acta Neuropathol 2018 Aug;136(2):273-291. Epub 2018 Mar 21 doi: 10.1007/s00401-018-1837-8. PMID: 29564591
Pilocytic astrocytoma: pathology, molecular mechanisms and markers.
Collins VP, Jones DT, Giannini C
Acta Neuropathol 2015 Jun;129(6):775-88. Epub 2015 Mar 20 doi: 10.1007/s00401-015-1410-7. PMID: 25792358Free PMC Article

Diagnosis

Pediatric Spinal Cord Diseases.
Lu VM, Niazi TN
Pediatr Rev 2021 Sep;42(9):486-499. doi: 10.1542/pir.2020-000661. PMID: 34470868
Pilocytic Astrocytoma: A Review of General, Clinical, and Molecular Characteristics.
Salles D, Laviola G, Malinverni ACM, Stávale JN
J Child Neurol 2020 Oct;35(12):852-858. Epub 2020 Jul 20 doi: 10.1177/0883073820937225. PMID: 32691644
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
Posterior Fossa Tumors.
Brandão LA, Young Poussaint T
Neuroimaging Clin N Am 2017 Feb;27(1):1-37. doi: 10.1016/j.nic.2016.08.001. PMID: 27889018
Pilocytic astrocytomas.
Bornhorst M, Frappaz D, Packer RJ
Handb Clin Neurol 2016;134:329-44. doi: 10.1016/B978-0-12-802997-8.00020-7. PMID: 26948364

Therapy

FIGHT-101, a first-in-human study of potent and selective FGFR 1-3 inhibitor pemigatinib in pan-cancer patients with FGF/FGFR alterations and advanced malignancies.
Subbiah V, Iannotti NO, Gutierrez M, Smith DC, Féliz L, Lihou CF, Tian C, Silverman IM, Ji T, Saleh M
Ann Oncol 2022 May;33(5):522-533. Epub 2022 Feb 14 doi: 10.1016/j.annonc.2022.02.001. PMID: 35176457
Dabrafenib plus trametinib in patients with BRAF(V600E)-mutant low-grade and high-grade glioma (ROAR): a multicentre, open-label, single-arm, phase 2, basket trial.
Wen PY, Stein A, van den Bent M, De Greve J, Wick A, de Vos FYFL, von Bubnoff N, van Linde ME, Lai A, Prager GW, Campone M, Fasolo A, Lopez-Martin JA, Kim TM, Mason WP, Hofheinz RD, Blay JY, Cho DC, Gazzah A, Pouessel D, Yachnin J, Boran A, Burgess P, Ilankumaran P, Gasal E, Subbiah V
Lancet Oncol 2022 Jan;23(1):53-64. Epub 2021 Nov 24 doi: 10.1016/S1470-2045(21)00578-7. PMID: 34838156
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free 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
BRAF Inhibition in BRAF(V600)-Mutant Gliomas: Results From the VE-BASKET Study.
Kaley T, Touat M, Subbiah V, Hollebecque A, Rodon J, Lockhart AC, Keedy V, Bielle F, Hofheinz RD, Joly F, Blay JY, Chau I, Puzanov I, Raje NS, Wolf J, DeAngelis LM, Makrutzki M, Riehl T, Pitcher B, Baselga J, Hyman DM
J Clin Oncol 2018 Dec 10;36(35):3477-3484. Epub 2018 Oct 23 doi: 10.1200/JCO.2018.78.9990. PMID: 30351999Free PMC Article

Prognosis

Pediatric Spinal Cord Diseases.
Lu VM, Niazi TN
Pediatr Rev 2021 Sep;42(9):486-499. doi: 10.1542/pir.2020-000661. PMID: 34470868
Adult pilocytic astrocytoma in the molecular era: a comprehensive review.
Gregory TA, Chumbley LB, Henson JW, Theeler BJ
CNS Oncol 2021 Mar 1;10(1):CNS68. Epub 2021 Jan 15 doi: 10.2217/cns-2020-0027. PMID: 33448230Free PMC Article
The use and efficacy of chemotherapy and radiotherapy in children and adults with pilocytic astrocytoma.
Parsons MW, Whipple NS, Poppe MM, Mendez JS, Cannon DM, Burt LM
J Neurooncol 2021 Jan;151(2):93-101. Epub 2020 Oct 31 doi: 10.1007/s11060-020-03653-y. PMID: 33131004
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
Pediatric Optic Pathway/Hypothalamic Glioma.
Aihara Y, Chiba K, Eguchi S, Amano K, Kawamata T
Neurol Med Chir (Tokyo) 2018 Jan 15;58(1):1-9. Epub 2017 Nov 9 doi: 10.2176/nmc.ra.2017-0081. PMID: 29118304Free PMC Article

Clinical prediction guides

Adult pilocytic astrocytoma in the molecular era: a comprehensive review.
Gregory TA, Chumbley LB, Henson JW, Theeler BJ
CNS Oncol 2021 Mar 1;10(1):CNS68. Epub 2021 Jan 15 doi: 10.2217/cns-2020-0027. PMID: 33448230Free PMC Article
The use and efficacy of chemotherapy and radiotherapy in children and adults with pilocytic astrocytoma.
Parsons MW, Whipple NS, Poppe MM, Mendez JS, Cannon DM, Burt LM
J Neurooncol 2021 Jan;151(2):93-101. Epub 2020 Oct 31 doi: 10.1007/s11060-020-03653-y. PMID: 33131004
Pilocytic Astrocytoma: A Review of General, Clinical, and Molecular Characteristics.
Salles D, Laviola G, Malinverni ACM, Stávale JN
J Child Neurol 2020 Oct;35(12):852-858. Epub 2020 Jul 20 doi: 10.1177/0883073820937225. PMID: 32691644
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free 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

Recent systematic reviews

Resection of brain lesions with a neuroendoscopic ultrasonic aspirator - a systematic literature review.
Ebel F, Greuter L, Guzman R, Soleman J
Neurosurg Rev 2022 Oct;45(5):3109-3118. Epub 2022 Jul 27 doi: 10.1007/s10143-022-01837-w. PMID: 35896917Free PMC Article
Psychiatric symptoms in children with low-grade glioma and craniopharyngioma: A systematic review.
Sherman SJ, Tanaka R, Qaddoumi I
J Psychiatr Res 2022 Apr;148:240-249. Epub 2022 Feb 2 doi: 10.1016/j.jpsychires.2022.01.056. PMID: 35149436
Meta-Analysis of Apparent Diffusion Coefficient in Pediatric Medulloblastoma, Ependymoma, and Pilocytic Astrocytoma.
Dury RJ, Lourdusamy A, Macarthur DC, Peet AC, Auer DP, Grundy RG, Dineen RA
J Magn Reson Imaging 2022 Jul;56(1):147-157. Epub 2021 Nov 29 doi: 10.1002/jmri.28007. PMID: 34842328
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
Adult Pilocytic Astrocytoma: An Institutional Series and Systematic Literature Review for Extent of Resection and Recurrence.
Bond KM, Hughes JD, Porter AL, Orina J, Fang S, Parney IF
World Neurosurg 2018 Feb;110:276-283. Epub 2017 Nov 24 doi: 10.1016/j.wneu.2017.11.102. PMID: 29180079

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