Intracranial ependymoma treatment

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Intracranial ependymoma treatment

The low prevalence of intracranial ependymoma in adults limits the ability to perform clinical trials. Therefore, treatment decisions are based on small, mostly retrospective studies and the role of chemotherapy has remained unclear.

Gross total resection (GTR) is considered the cornerstone of therapy 1) 2).

Adjuvant radiotherapy is often delivered to improve long-term disease control. Over the years, radiation treatment for ependymoma has evolved from cranio spinal radiation (CSI) 3) 4) to focal radiation 5) 6)

The major consensus was reached that treatment decisions for ependymoma (outside of clinical trials) should not be based on grading (II vs III). Supratentorial and posterior fossa ependymomas are distinct diseases, although the impact on therapy is still evolving. Molecular subgrouping should be part of all clinical trials henceforth 7).

Stereotactic Radiosurgery

Seven centers participating in the International Radiosurgery Research Foundation identified 89 intracranial ependymoma patients who underwent SRS (113 tumors). The median patient age was 16.3 yr (2.9-80). All patients underwent previous surgical resection and radiation therapy (RT) of their ependymomas and 40 underwent previous chemotherapy. Grade 2 ependymomas were present in 42 patients (52 tumors) and grade 3 ependymomas in 48 patients (61 tumors). The median tumor volume was 2.2 cc (0.03-36.8) and the median margin dose was 15 Gy (9-24).

Forty-seven (53%) patients were alive and 42 (47%) patients died at the last follow-up. The overall survival after SRS was 86% at 1 yr, 50% at 3 yr, and 44% at 5 yr. Smaller total tumor volume was associated with longer overall survival (P = .006). Twenty-two patients (grade 2: n = 9, grade 3: n = 13) developed additional recurrent ependymomas in the craniospinal axis. The progression-free survival after SRS was 71% at 1 yr, 56% at 3 yr, and 48% at 5 yr. Adult age, female sex, and smaller tumor volume indicated significantly better progression-free survival. Symptomatic adverse radiation effects were seen in 7 patients (8%).

SRS provides another management option for residual or recurrent progressive intracranial ependymoma patients who have failed initial surgery and RT 8).

Chemotherapy

see Chemotherapy for intracranial ependymoma.

References

1)

Gondi V, Vogelbaum MA, Grimm S, Mehta MP. Primary intracranial neoplasm. In: Halperin EC, Wazer DE, Perez CA, Brady LW, editors. Perez and Brady’s Principles and Practice of Radiation Oncology. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2013. pp. 669–70.
2)

Freeman CR, Farmer JP, Taylor RE. Central Nervous System Tumors in Children. In: Halperin EC, Wazer DE, Perez CA, Brady LW, editors. Perez and Brady’s Principles and Practice of Radiation Oncology. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2013. pp. 1643–5.
3)

Salazar OM, Castro-Vita H, VanHoutte P, Rubin P, Aygun C. Improved survival in cases of intracranial ependymoma after radiation therapy. Late report and recommendations. J Neurosurg. 1983;59:652–9.
4)

Goldwein JW, Corn BW, Finlay JL, Packer RJ, Rorke LB, Schut L. Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer. 1991;67:2766–71.
5)

Paulino AC. The local field in infratentorial ependymoma: Does the entire posterior fossa need to be treated? Int J Radiat Oncol Biol Phys. 2001;49:757–61.
6)

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;10:258–66.
7)

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.
8)

Kano H, Su YH, Wu HM, Simonova G, Liscak R, Cohen-Inbar O, Sheehan JP, Meola A, Sharma M, Barnett GH, Mathieu D, Vasas LT, Kaufmann AM, Jacobs RC, Lunsford LD. Stereotactic Radiosurgery for Intracranial Ependymomas: An International Multicenter Study. Neurosurgery. 2019 Jan 1;84(1):227-234. doi: 10.1093/neuros/nyy082. PubMed PMID: 29608701.

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