international survey on management of paediatric ependymomas : preliminary results
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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. Introduction. - PowerPoint PPT PresentationTRANSCRIPT
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