1. International Survey on Management of Paediatric Ependymomas: Preliminary Results Guirish Solanki ¥, G Narenthiran § Department of Neurosurgery ¥ Birmingham Children’s Hospital & § Wessex Neurological Centre, Southampton, UK

2. Introduction Improved survival in most paediatric brain tumours developments in diagnostic imaging Improved operative micro-neurosurgical techniques improved delivery of chemotherapy and radiotherapy provision of supportive care. Some tumours remain mainly "surgical" lesions role of adjuvant chemotherapy remains controversial in some situations.

3. Ependymomas in the UK Make up about 10% of brain tumours Majority found in the posterior fossa Over 50% are below the age of 5 years. The use of radiotherapy is limited in a significant proportion of children under 3 years of age – Gross Total Resection or – Subtotal Resection for those.

4. Ependymomas in the UK In England Number of new cases per year is small =30- 35 cases Large studies of significance take time. Infants and children under 3 years of age Tendency to use chemotherapy in the UK This is not universally accepted.

5. The International Survey: Aims To understand current practice – Extent of resection – Use of chemotherapy – Timing of radiotherapy Anonymity of individual or unit optional Report the findings to the International Neurosurgical Online Conference members

6. The International Survey: Definitions Gross Total Resection: Radical curative resection with no visible residual tumour Sometimes unclear if there is a residue or surgical change < less than 15mm 2 on post-op MRI scan accepted Subtotal Resection: Resection with residual tumour visible at end of surgery more than 1.5 cm square on post-op MRI scan Second look Surgery: When initial surgery was incomplete, a second elective surgical procedure to allow a more complete tumour clearance.

7. The International Survey: Caveats The questions apply to – supratentorial (ST) and infratentorial(PF) ependymomas – did not include primary spinal tumours. The presence of spinal metastasis – Not an exclusion for reporting treatment strategy – Management of PF or ST lesions was allowed within this survey and details entered in additional comments for each section.

8. The International Survey: Methods An online survey was made available to members of the neurosurgery academic mailing list International Paediatric Units were also sent the survey by email to increase yield. – Not an exhaustive global list Data was collected and analysed using a standard spreadsheet and analysis package

9. The International Survey: Respondent and Practice status Name Position – (Trainee; Consultant; Senior Lecturer; Professor; Clinical Lead; Head of Department) Practice details – Mainly adult neurosurgeon (75% adult practice) – Mainly paediatric neurosurgeon(75% paediatric practice) – Combined practice(50-50) Institution & Type – Public/Private ± Academic Address – City/Country/Email /Tel

10. Case Load & Location How many paediatric ependymoma cases do you treat per year in your unit? – Between 1 -5 new cases per year – Between 6-10 new cases per year – More than 10 new cases per year How many are in the posterior fossa? – <50% – 50-75% – >75%

11. Surgical Strategy Aggressive Radical Curative Resection attempting curative resection – (GTR accepting cranial nerve deficits, hemiparesis, cerebellar/brainstem dysfunction as collateral damage in return for a better chance of cure) Resection attempting curative resection – (GTR only if possible without significant neurological deficits, accepting residual lesion with better quality of life)

12. Extent of Resection: GRT/STR With regards to extent of primary resection: – Achieved GTR in >95% ; – Achieved GTR in 76%-95%; – Achieved GTR in 61%-75%; – Achieved GTR in 50-60%; – Achieved GTR in < 50% of cases

13. Adjuvant Therapy following Primary Resection Posterior fossa under and over 3 years of age (GTR or with STR) – no adjuvant therapy – Chemotherapy – Conformal radiotherapy; – Craniospinal radiotherapy Any age In Supratentorial Ependymoma – Resection + no adjuvant therapy – Resection + Chemotherapy followed by radiotherapy over 3 years of age – Resection + radiotherapy over 3 years of age

14. Managing Residual/Recurrent Tumours Under and over 3 years of age – second look surgery alone – Chemotherapy + second look surgery or – second look surgery + chemotherapy – Second look surgery and conformal / local radiotherapy – second look surgery and craniospinal radiotherapy – Some other therapeutic option (please specify)

15. Results: Workload Incidence in most units is between 1-5 new cases per year. Few centres do >10 new cases/year.

16. Results: Surgical Strategy

17. Results: Extent of Resection

18. Results: Surgical Strategy & Extent of Resection

19. A surgical strategy of curative resection attempting to preserve function is preferred. More units perform radical resection now. Surgical strategy is not uniform and varies geographycally Most units report GTR in 60-95% of cases but some units have GTR in >95%.

20. Adjuvant therapy under age of 3

21. Adjuvant Therapy under 3 years

22. Results: Chemotherapy In children under 3 years adjuvant chemotherapy is given. An increasing number of units use chemotherapy prior to second look surgery following recurrence or surgery for residual disease. Adjuvant therapy mode of delivery varies between units. This variation is greater geographically.

23. Results: Radiotherapy Radiotherapy is generally accepted as adjuvant therapy for children above 3 years.

24. Discussion: Extent of surgical resection The most consistent prognostic factor for cure is extent of resection Recent reports suggest improved outcomes with a more radical resection strategy and that radiotherapy is given in children under 18 months aiming at improving survival and cure. Is there an ethical compulsion to treat all costs to save a life? Radical surgical resection Multiple surgical resections. How much is enough? Radiotherapy below 3 years of age

25. Discussion: Radiotherapy Radiation therapy – the standard adjuvant treatment, – Need data comparing surgery alone to surgery and postoperative radiotherapy Craniospinal irradiation – used in the past to treat these tumors – most common pattern of failure is isolated local relapse. – prophylactic spinal irradiation does not prevent spinal dissemination. – Conformal radiotherapy is preferred. – Hyperfractionated radiotherapy doses of greater than 65 Gy may improve progression-free survival for subtotal resections.

26. Role of chemotherapy Tumour control until safe to give Radiotherapy under 3 yrs Prelude to further resection any age Adjuvant to radiotherapy (any age) Role in older children needs further clarification Discussion: Chemotherapy

27. Conclusion This is only Preliminary data. 48 units so far. Most units surveyed in Europe do 1-5 cases/year. The US respondents report greater numbers/unit (> 6 cases /year) Greater caseload related to more radical surgery. More radical surgery related to greater extent of resection Radiotherapy under the age of 3 years more prevalent in the US Chemotherapy under the age of 3 more prevalent in Europe A consensus is required on optimal therapy for childhood ependymomas.

28. Acknowledgements The Academic Neurosurgery Online Group for hosting the survey. Respondents for taking the time to contribute.

29. THANK YOU