Anaplastic ependymoma treatment [Neurosurgery Wiki]

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====== Anaplastic ependymoma treatment ======

The standard of care for ependymoma varies by geographic location and treatment center. Despite differing opinions about potential indications and clinical scenarios of ependymoma, experts worldwide generally agree that surgery and radiation therapy (RT) are mainstays of treatment
((Pajtler KW, Mack SC, Ramaswamy V, Smith CA, Witt H, Smith A, Hansford JR, von 
Hoff K, Wright KD, Hwang E, Frappaz D, Kanemura Y, Massimino M, Faure-Conter C,
Modena P, Tabori U, Warren KE, Holland EC, Ichimura K, Giangaspero F, Castel D,
von Deimling A, Kool M, Dirks PB, Grundy RG, Foreman NK, Gajjar A, Korshunov A,
Finlay J, Gilbertson RJ, Ellison DW, Aldape KD, Merchant TE, Bouffet E, Pfister
SM, Taylor MD. The current consensus on the clinical management of intracranial
ependymoma and its distinct molecular variants. Acta Neuropathol. 2017
Jan;133(1):5-12. doi: 10.1007/s00401-016-1643-0. Epub 2016 Nov 17. PubMed PMID:
27858204; PubMed Central PMCID: PMC5209402.
)).

Surgery and RT form the backbone for Children’s Oncology Group (COG)
((Merchant TE: Observation or radiation therapy and/or chemotherapy and second surgery in treating children who have undergone surgery for ependymoma (ClinicalTrials.gov Identifier: NCT00027846). https://clinicaltrials.gov/ct2/show/NCT00027846?term=nct00027846&rank=1. 2001.))
((Smith AA: Maintenance chemotherapy or observation following induction chemotherapy and radiation therapy in treating younger patients with newly diagnosed ependymoma (ClinicalTrials.gov Identifier: NCT01096368). https://clinicaltrials.gov/ct2/show/NCT01096368?term=nct01096368&rank=1. 2010.))
and International Society for Pediatric Oncology (SIOP)
((Frappaz D: An international clinical program for the diagnosis and treatment of children with ependymoma (SIOP-EP-II) (ClinicalTrials.gov Identifier: NCT02265770). https://clinicaltrials.gov/ct2/show/NCT02265770?term=nct02265770&rank=1. 2014))
trials on ependymoma.

Surgery and RT were established as the gold standard for intracranial ependymoma, as outlined in a benchmark study at St Jude Children’s Research Hospital (St Jude) from 1997 to 2007
((Merchant TE, Li C, Xiong X, Kun LE, Boop FA, Sanford RA. Conformal
radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet
Oncol. 2009 Mar;10(3):258-66. PubMed PMID: 19274783; PubMed Central PMCID:
PMC3615425.
))

The benefit of postoperative chemotherapy for anaplastic [[ependymoma]] remains unknown.

[[Temozolomide]] (TMZ) may be effective for pediatric refractory anaplastic ependymoma with low [[MGMT]] protein expression
((Komori K, Yanagisawa R, Miyairi Y, Sakashita K, Shiohara M, Fujihara I, Morita
D, Nakamura T, Ogiso Y, Sano K, Shirahata M, Fukuoka K, Ichimura K, Shigeta H.
Temozolomide treatment for pediatric refractory anaplastic ependymoma with low
MGMT protein expression. Pediatr Blood Cancer. 2015 Aug 25. doi:
10.1002/pbc.25696. [Epub ahead of print] PubMed PMID: 26305586.)).
----
The need to diagnose and differentiate the aggressive variants, which include the [[Posterior fossa type A ependymoma]] and the supratentorial [[Ependymoma RELA fusion positive]], is imperative to escalate therapy and improve survival
((Khatua S, Magnum R, Bertrand KC, Zaky W, McCall D, Mack SC. Pediatric
ependymoma: current treatment and newer therapeutic insights. Future Oncol. 2018 
Nov 12. doi: 10.2217/fon-2018-0502. [Epub ahead of print] PubMed PMID: 30418040.
)).