International Survey on Management of Paediatric Ependymomas:

Preliminary Results

Guirish SolankiGuirish Solanki¥¥, G Narenthiran, G Narenthiran§§

Department of Neurosurgery¥ Birmingham Children’s Hospital &

§ Wessex Neurological Centre, Southampton, UK

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.

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.

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.

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

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.

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.

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

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

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%

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)

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

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

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)

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

Results: Surgical Strategy

Results: Extent of Resection

Results: Surgical Strategy & Extent of Resection

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%.

Adjuvant therapy under age of 3

Adjuvant Therapy under 3 years

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.

Results: Radiotherapy

• Radiotherapy is generally accepted as adjuvant therapy for children above 3 years.

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

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.

• 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

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.

Acknowledgements

• The Academic Neurosurgery Online Group for hosting the survey.

• Respondents for taking the time to contribute.

THANK YOU