International Survey on Management of Paediatric Ependymomas : Preliminary Results

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 15mm2 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. Results: Surgical Strategy & Extent of Resection • 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. Discussion: Chemotherapy • 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

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