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Pilomyxoid astrocytoma

MedGen UID:
276757
•Concept ID:
C1519086
•
Neoplastic Process
Synonyms: Pilomyxoid Astrocytoma; pilomyxoid astrocytoma; PMA
SNOMED CT: Pilomyxoid astrocytoma (388600004); Pilomyxoid astrocytoma (1157070004)
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)
 
Monarch Initiative: MONDO:0016692
Orphanet: ORPHA251615

Definition

An astrocytic tumor of uncertain relation to pilocytic astrocytoma. It occurs predominantly in infants and young children. It is characterized by a monomorphic architectural pattern, usually associated with the absence of Rosenthal fibers and eosinophilic granular bodies. The clinical course is usually aggressive. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPilomyxoid astrocytoma
Follow this link to review classifications for Pilomyxoid astrocytoma in Orphanet.

Professional guidelines

PubMed

Targeted fusion analysis can aid in the classification and treatment of pediatric glioma, ependymoma, and glioneuronal tumors.
Lake JA, Donson AM, Prince E, Davies KD, Nellan A, Green AL, Mulcahy Levy J, Dorris K, Vibhakar R, Hankinson TC, Foreman NK, Ewalt MD, Kleinschmidt-DeMasters BK, Hoffman LM, Gilani A
Pediatr Blood Cancer 2020 Jan;67(1):e28028. Epub 2019 Oct 8 doi: 10.1002/pbc.28028. PMID: 31595628Free PMC Article
Successful treatment with a low-dose cisplatin--etoposide regimen for patients with diencephalic syndrome.
Sardi I, Bresci C, Schiavello E, Biassoni V, Fratoni V, Cardellicchio S, Genitori L, Aricò M, Massimino M
J Neurooncol 2012 Sep;109(2):375-83. Epub 2012 Jun 21 doi: 10.1007/s11060-012-0903-7. PMID: 22717669
Pilomyxoid astrocytoma: diagnosis, prognosis, and management.
Komotar RJ, Mocco J, Jones JE, Zacharia BE, Tihan T, Feldstein NA, Anderson RC
Neurosurg Focus 2005 Jun 15;18(6A):E7. PMID: 16048293

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
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Heterogeneity of histopathological presentation of pilocytic astrocytoma - diagnostic pitfalls. A review.
Matyja E, Grajkowska W, Stępień K, Naganska E
Folia Neuropathol 2016;54(3):197-211. doi: 10.5114/fn.2016.62530. PMID: 27764513
T2 and DWI in pilocytic and pilomyxoid astrocytoma with pathologic correlation.
Horger M, Vogel MN, Beschorner R, Ernemann U, Wörner J, Fenchel M, Ebner F, Nägele T, Heckl S
Can J Neurol Sci 2012 Jul;39(4):491-8. doi: 10.1017/s0317167100014013. PMID: 22728857
The 2007 WHO classification of tumours of the central nervous system.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P
Acta Neuropathol 2007 Aug;114(2):97-109. Epub 2007 Jul 6 doi: 10.1007/s00401-007-0243-4. PMID: 17618441Free PMC Article

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
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Heterogeneity of histopathological presentation of pilocytic astrocytoma - diagnostic pitfalls. A review.
Matyja E, Grajkowska W, Stępień K, Naganska E
Folia Neuropathol 2016;54(3):197-211. doi: 10.5114/fn.2016.62530. PMID: 27764513
Posterior fossa tumors in infants and neonates.
Spennato P, Nicosia G, Quaglietta L, Donofrio V, Mirone G, Di Martino G, Guadagno E, del Basso de Caro ML, Cascone D, Cinalli G
Childs Nerv Syst 2015 Oct;31(10):1751-72. Epub 2015 Sep 9 doi: 10.1007/s00381-015-2783-6. PMID: 26351228
The molecular biology of WHO grade II gliomas.
Marko NF, Weil RJ
Neurosurg Focus 2013 Feb;34(2):E1. doi: 10.3171/2012.12.FOCUS12283. PMID: 23373446

Therapy

Postoperative speech impairment and surgical approach to posterior fossa tumours in children: a prospective European multicentre cohort study.
Grønbæk JK, Wibroe M, Toescu S, Frič R, Thomsen BL, Møller LN, Grillner P, Gustavsson B, Mallucci C, Aquilina K, Fellows GA, Molinari E, Hjort MA, Westerholm-Ormio M, Kiudeliene R, Mudra K, Hauser P, van Baarsen K, Hoving E, Zipfel J, Nysom K, Schmiegelow K, Sehested A, Juhler M, Mathiasen R; CMS study group
Lancet Child Adolesc Health 2021 Nov;5(11):814-824. Epub 2021 Oct 6 doi: 10.1016/S2352-4642(21)00274-1. PMID: 34624241
Bevacizumab Therapy for Pilomyxoid Astrocytoma.
Metts RD, Bartynski W, Welsh CT, Kinsman S, Bredlau AL
J Pediatr Hematol Oncol 2017 May;39(4):e219-e223. doi: 10.1097/MPH.0000000000000824. PMID: 28338567
The role of surgery in optic pathway/hypothalamic gliomas in children.
Goodden J, Pizer B, Pettorini B, Williams D, Blair J, Didi M, Thorp N, Mallucci C
J Neurosurg Pediatr 2014 Jan;13(1):1-12. Epub 2013 Oct 18 doi: 10.3171/2013.8.PEDS12546. PMID: 24138145
Successful treatment with a low-dose cisplatin--etoposide regimen for patients with diencephalic syndrome.
Sardi I, Bresci C, Schiavello E, Biassoni V, Fratoni V, Cardellicchio S, Genitori L, Aricò M, Massimino M
J Neurooncol 2012 Sep;109(2):375-83. Epub 2012 Jun 21 doi: 10.1007/s11060-012-0903-7. PMID: 22717669
A lower-dose, lower-toxicity cisplatin-etoposide regimen for childhood progressive low-grade glioma.
Massimino M, Spreafico F, Riva D, Biassoni V, Poggi G, Solero C, Gandola L, Genitori L, Modena P, Simonetti F, Potepan P, Casanova M, Meazza C, Clerici CA, Catania S, Sardi I, Giangaspero F
J Neurooncol 2010 Oct;100(1):65-71. Epub 2010 Feb 12 doi: 10.1007/s11060-010-0136-6. PMID: 20151174

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
Pilomyxoid astrocytomas: a short review.
Kulac I, Tihan T
Brain Tumor Pathol 2019 Apr;36(2):52-55. Epub 2019 Apr 3 doi: 10.1007/s10014-019-00343-0. PMID: 30945015
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
The molecular biology of WHO grade II gliomas.
Marko NF, Weil RJ
Neurosurg Focus 2013 Feb;34(2):E1. doi: 10.3171/2012.12.FOCUS12283. PMID: 23373446
A lower-dose, lower-toxicity cisplatin-etoposide regimen for childhood progressive low-grade glioma.
Massimino M, Spreafico F, Riva D, Biassoni V, Poggi G, Solero C, Gandola L, Genitori L, Modena P, Simonetti F, Potepan P, Casanova M, Meazza C, Clerici CA, Catania S, Sardi I, Giangaspero F
J Neurooncol 2010 Oct;100(1):65-71. Epub 2010 Feb 12 doi: 10.1007/s11060-010-0136-6. PMID: 20151174

Clinical prediction guides

Molecular Alterations in Pediatric Low-Grade Gliomas That Led to Death.
Ahrendsen JT, Sinai C, Meredith DM, Malinowski SW, Cooney TM, Bandopadhayay P, Ligon KL, Alexandrescu S
J Neuropathol Exp Neurol 2021 Nov 19;80(11):1052–1059. Epub 2021 Sep 27 doi: 10.1093/jnen/nlab097. PMID: 34580728
Spectroscopic measurement of 5-ALA-induced intracellular protoporphyrin IX in pediatric brain tumors.
Schwake M, Kaneko S, Suero Molina E, Müther M, Schipmann S, Köchling M, Brentrup A, Stummer W
Acta Neurochir (Wien) 2019 Oct;161(10):2099-2105. Epub 2019 Aug 21 doi: 10.1007/s00701-019-04039-4. PMID: 31435824
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
The molecular biology of WHO grade II gliomas.
Marko NF, Weil RJ
Neurosurg Focus 2013 Feb;34(2):E1. doi: 10.3171/2012.12.FOCUS12283. PMID: 23373446
Pilomyxoid astrocytoma.
Azad S, Kudesia S, Chawla N, Azad R, Singhal M, Rai SM, Arora P
Indian J Pathol Microbiol 2010 Apr-Jun;53(2):294-6. doi: 10.4103/0377-4929.64283. PMID: 20551536

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