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Ependymoma in children less than 3 year of age

Ependymoma in children less than 3 year of age. Sidnei Epelman , M.D. SIOP - Sao Paulo, Brazil . October 2009 epelman@inctrbrasil.org. Epidemiology of Ependymoma. 2-2.5 children per million in Europe and North America Males slightly more common

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Ependymoma in children less than 3 year of age

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  1. Ependymoma in children less than 3 year of age Sidnei Epelman, M.D. SIOP - Sao Paulo, Brazil. October 2009 epelman@inctrbrasil.org

  2. EpidemiologyofEpendymoma • 2-2.5 children per million in Europe and North America • Males slightly more common • Over half occur in children <5 years of age at diagnosis • 6 to 10% of all intracranial childhood tumors • Effective treatment remains difficult task • Success measure • Event free or overall survival • Potential to irreversible damage to the brain

  3. Epidemiology

  4. Ependymoma in Younger ChildrenPresentation • In Infants: • Enlarging head circumference • Emesis, weight loss/poor weight gain • In older children: • Classical symptoms/signs pertinent to location

  5. EpendymomaFactors affecting outcome • Histological grade based WHO classification • Biomolecular studies • gain of 1q25 – poor • EGFR overexpression - poor • Low express of nucleolin – favorable

  6. Role ofChemotherapy - controvertial Reports – betterresectionwith use ofpre-operativechemotherapy Complete resection – prognosticfactor Anaplastic tumor – impact in survival? Supratentorialandnotanaplastic – surgery is curative EpendymomaTreatment

  7. Surgical Management of Intra-Cranial Ependymomas • Radical surgical resection is associated with best survival outcome - irrespective of age, site, pathology or other treatment (irradiation and/or chemotherapy): • Gross total resection(GTR) versus non gross total resection: 60-89% versus 21-46% (5 year OS) • Surgery alone may be associated with a high cure rate for supratentorial, parenchymally based ependymomas.

  8. Radiation Therapy for Intra-Cranial Ependymomas • The overwhelming majority of patients present - and recur - with localized disease. • Focal conformal irradiation -not craniospinal or whole brain - is the accepted standard of treatment - at doses in excess of 4500cGy. • Newer technologies (FSRT, IMRT, Protons, Gamma or Cyber Knife) may permit tumor control with less morbidity even in the youngest of children.

  9. RadiationTherapy: Late Effects • Unacceptable late cognitive deficits of irradiation for supratentorial tumors – depending upon location • Unknown late cognitive deficits for the youngest of children with infratentorial tumors • Clearly established second malignancy rate approaching 20-50% at 20 years in young children irradiated for medulloblastoma and CNS germinoma

  10. Chemotherapy in Younger Children with Ependymoma • Ependymoma are associated with late recurrences • Young children who relapse do eventually die, despite often many years of survival with repeat surgery,+/-RT - thus, OS data are important after very long follow-up

  11. Young Children (<3-5yo) with Ependymomas: “Chemotherapy only” Protocols

  12. Ependymoma Head Start Response to induction chemotherapy One toxic death during induction

  13. Chemotherapy Strategies for Younger Children with Ependymoma • Brief intensivechemotherapy-only strategies (eg. Head Start ) have poorer EFS than less dose-intensive regimens of longer duration • Young children with measurable tumor do respond to chemotherapy (20% CR on Head Start without HD-MTX) but not sustained

  14. Chemotherapy Strategies for Younger Children with Ependymoma • No chemotherapy-only regimens to date produce as good EFS as the most recent XRT only studies (St.Jude) – but follow-up of EFS and cognitive outcome still too early! • Delayed radiotherapy strategies (POG, HIT) appear to produce poorer EFS than immediate radiotherapy strategies.

  15. Primary postoperative chemotherapy without Rxt • 89 patients < 3 years • 50/80 non metastatic progressed – 34 irradiated for progression • 5 year freedom from Rxt – 42% • Overall survival at 3y 79% and at 5y 63% • 80% progressed – only local

  16. Primary postoperative chemotherapy without Rxt • No significant difference in EFS and OS • Complete and incomplete resection • Histological grade • Age at diagnosis • Site of disease • 23 highest relative dose intensity – highest 5-year overall survival of 76% x 52% with lowest relative dose intensity of chemotherapy

  17. Primary postoperative chemotherapy without Rxt

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