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 ¹⁾.

Surgery and RT form the backbone for Children’s Oncology Group (COG) ²⁾ ³⁾ and International Society for Pediatric Oncology (SIOP) ⁴⁾ 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 ⁵⁾

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 ⁶⁾.

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 ⁷⁾.

¹⁾

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.

²⁾

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.

³⁾

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.

⁴⁾

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

⁵⁾

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.

⁶⁾

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.

⁷⁾

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.