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Pediatric intracranial ependymoma: the roles of surgery, radiation and chemotherapy

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Abstract

Management of pediatric intracranial ependymomas poses a major challenge, and optimal treatment remains controversial. We sought to investigate the roles of surgery, radiation, and chemotherapy in a historical cohort. Thirty-nine children, age 21 or younger, with non-metastatic intracranial ependymomas were treated from 1972 to 2008. Median age was 8 years (range 0.2–19.1). Twenty-one patients (54%) underwent GTRs, and 18 (45%) underwent STRs. Twenty-six patients (67%) received upfront adjuvant RT (67%), and 14 (44%) received adjuvant chemotherapy. Twenty-four patients had disease recurrence and 12 died. Only one patient recurred after 5 years. Median PFS was 2.7 years and median OS was 20 years. Fifteen year PFS and OS were 30 and 67%. Adjuvant RT was associated with improved PFS (P = 0.045), and remained significant after adjusting for EOR (P = 0.04). Greater EOR trended towards prolonged survival, but did not reach statistical significance (P = 0.156). Of the patients that underwent GTR, the median PFS was 38 months for those treated with adjuvant RT versus 30 months for those that were not treated with RT. Of the patients that had STR, the median PFS for those treated with RT was 26.3 months versus 6.9 months for those were not treated with RT. In conclusion, for localized intracranial pediatric ependymomas, adjuvant RT is associated with improved PFS, even after adjusting for EOR. Our findings suggest the benefit of RT even in the presence of GTR. Future prospective studies with larger sample number are needed to validate our findings.

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Abbreviations

PFS:

Progression-free survival

OS:

Overall survival

EOR:

Extent of resection

GTR:

Gross total resection

STR:

Subtotal resection

RT:

Radiation therapy

NED:

No evidence of disease

CI:

Confidence interval

PH:

Proportional hazards

POG:

Pediatric Oncology Group

CCG:

Children’s Oncology Group

CT:

Computed tomography

MRI:

Magnetic resonance imaging

WHO:

World Health Organization

CNS:

Central nervous system

CSI:

Craniospinal irradiation

3DCRT:

3 Dimensional conformal radiation therapy

IMRT:

Intensity modulated radiation therapy

References

  1. Goldwein JW, Leahy JM, Packer RJ, Sutton LN, Curran WJ, Rorke LB et al (1990) Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys 19:1497–1502

    Article  PubMed  CAS  Google Scholar 

  2. Miller RW, Young JL Jr, Novakovic B (1995) Childhood cancer. Cancer 75:395–405

    Article  PubMed  CAS  Google Scholar 

  3. Rousseau P, Habrand JL, Sarrazin D, Kalifa C, Terrier-Lacombe MJ, Rekacewicz C et al (1994) Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386

    Article  PubMed  CAS  Google Scholar 

  4. Vanuytsel LJ, Bessell EM, Ashley SE, Bloom HJ, Brada M (1992) Intracranial ependymoma: long-term results of a policy of surgery and radiotherapy. Int J Radiat Oncol Biol Phys 23:313–319

    Article  PubMed  CAS  Google Scholar 

  5. Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J et al (1999) A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21:203–211

    Article  PubMed  CAS  Google Scholar 

  6. Merchant TE, Haida T, Wang MH, Finlay JL, Leibel SA (1997) Anaplastic ependymoma: treatment of pediatric patients with or without craniospinal radiation therapy. J Neurosurg 86:943–949

    Article  PubMed  CAS  Google Scholar 

  7. Merchant TE, Mulhern RK, Krasin MJ, Kun LE, Williams T, Li C et al (2004) Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol 22:3156–3162

    Article  PubMed  Google Scholar 

  8. Carrie C, Mottolese C, Bouffet E, Negrier S, Bachelot TH, Lasset C et al (1995) Non-metastatic childhood ependymomas. Radiother Oncol 36:101–106

    Article  PubMed  CAS  Google Scholar 

  9. Perilongo G, Massimino M, Sotti G, Belfontali T, Masiero L, Rigobello L et al (1997) Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-oncology Group. Med Pediatr Oncol 29:79–85

    Article  PubMed  CAS  Google Scholar 

  10. Pollack IF, Gerszten PC, Martinez AJ, Lo KH, Shultz B, Albright AL et al (1995) Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37:655–666

    Article  PubMed  CAS  Google Scholar 

  11. Timmermann B, Kortmann RD, Kuhl J, Meisner C, Slavc I, Pietsch T et al (2000) Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: results of the German prospective trials HIT 88/89 and HIT 91. Int J Radiat Oncol Biol Phys 46:287–295

    Article  PubMed  CAS  Google Scholar 

  12. Shu HK, Sall WF, Maity A, Tochner ZA, Janss AJ, Belasco JB et al (2007) Childhood intracranial ependymoma: twenty-year experience from a single institution. Cancer 110:432–441

    Article  PubMed  Google Scholar 

  13. Mansur DB, Perry A, Rajaram V, Michalski JM, Park TS, Leonard JR et al (2005) Postoperative radiation therapy for grade II and III intracranial ependymoma. Int J Radiat Oncol Biol Phys 61:387–391

    Article  PubMed  Google Scholar 

  14. Jaing TH, Wang HS, Tsay PK, Tseng CK, Jung SM, Lin KL et al (2004) Multivariate analysis of clinical prognostic factors in children with intracranial ependymomas. J Neurooncol 68:255–261

    Article  PubMed  Google Scholar 

  15. Merchant TE, Jenkins JJ, Burger PC, Sanford RA, Sherwood SH, Jones-Wallace D et al (2002) Influence of tumor grade on time to progression after irradiation for localized ependymoma in children. Int J Radiat Oncol Biol Phys 53:52–57

    Article  PubMed  Google Scholar 

  16. Sutton LN, Goldwein J, Perilongo G, Lang B, Schut L, Rorke L et al (1990) Prognostic factors in childhood ependymomas. Pediatr Neurosurg 16:57–65

    Article  PubMed  Google Scholar 

  17. Paulino AC, Wen BC, Buatti JM, Hussey DH, Zhen WK, Mayr NA et al (2002) Intracranial ependymomas: an analysis of prognostic factors and patterns of failure. Am J Clin Oncol 25:117–122

    Article  PubMed  Google Scholar 

  18. Mork SJ, Loken AC (1977) Ependymoma: a follow-up study of 101 cases. Cancer 40:907–915

    Article  PubMed  CAS  Google Scholar 

  19. Rosenblum MK (1998) Ependymal tumors: a review of their diagnostic surgical pathology. Pediatr Neurosurg 28:160–165

    Article  PubMed  CAS  Google Scholar 

  20. Hukin J, Epstein F, Lefton D, Allen J (1998) Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29:40–45

    Article  PubMed  CAS  Google Scholar 

  21. Paulino AC (2001) The local field in infratentorial ependymoma: does the entire posterior fossa need to be treated? Int J Radiat Oncol Biol Phys 49:757–761

    Article  PubMed  CAS  Google Scholar 

  22. Merchant TE, Fouladi M (2005) Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neurooncol 75:287–299

    Article  PubMed  Google Scholar 

  23. Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD (1987) Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Biol Phys 13:1457–1462

    Article  PubMed  CAS  Google Scholar 

  24. Paulino AC, Wen BC (2000) The significance of radiotherapy treatment duration in intracranial ependymoma. Int J Radiat Oncol Biol Phys 47:585–589

    Article  PubMed  CAS  Google Scholar 

  25. Massimino M, Gandola L, Giangaspero F, Sandri A, Valagussa P, Perilongo G et al (2004) Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study. Int J Radiat Oncol Biol Phys 58:1336–1345

    Article  PubMed  Google Scholar 

  26. Duffner PK, Krischer JP, Sanford RA, Horowitz ME, Burger PC, Cohen ME et al (1998) Prognostic factors in infants and very young children with intracranial ependymomas. Pediatr Neurosurg 28:215–222

    Article  PubMed  CAS  Google Scholar 

  27. Robertson PL, Zeltzer PM, Boyett JM, Rorke LB, Allen JC, Geyer JR et al (1998) Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children’s Cancer Group. J Neurosurg 88:695–703

    Article  PubMed  CAS  Google Scholar 

  28. McGuire CS, Sainani KL, Fisher PG (2009) Both location and age predict survival in ependymoma: a SEER study. Pediatr Blood Cancer 52:65–69

    Article  PubMed  Google Scholar 

  29. Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417

    Article  PubMed  CAS  Google Scholar 

  30. Wallner KE, Wara WM, Sheline GE, Davis RL (1986) Intracranial ependymomas: results of treatment with partial or whole brain irradiation without spinal irradiation. Int J Radiat Oncol Biol Phys 12:1937–1941

    Article  PubMed  CAS  Google Scholar 

  31. West CR, Bruce DA, Duffner PK (1985) Ependymomas. Factors in clinical and diagnostic staging. Cancer 56:1812–1816

    Article  PubMed  CAS  Google Scholar 

  32. Foreman NK, Love S, Thorne R (1996) Intracranial ependymomas: analysis of prognostic factors in a population-based series. Pediatr Neurosurg 24:119–125

    Article  PubMed  CAS  Google Scholar 

  33. Figarella-Branger D, Civatte M, Bouvier-Labit C, Gouvernet J, Gambarelli D, Gentet JC et al (2000) Prognostic factors in intracranial ependymomas in children. J Neurosurg 93:605–613

    Article  PubMed  CAS  Google Scholar 

  34. Tihan T, Zhou T, Holmes E, Burger PC, Ozuysal S, Rushing EJ (2008) The prognostic value of histological grading of posterior fossa ependymomas in children: a Children’s Oncology Group study and a review of prognostic factors. Mod Pathol 21:165–177

    PubMed  Google Scholar 

  35. Foreman NK, Love S, Gill SS, Coakham HB (1997) Second-look surgery for incompletely resected fourth ventricle ependymomas: technical case report. Neurosurgery 40:856–860

    Article  PubMed  CAS  Google Scholar 

  36. Chakraborty A, Harkness W, Phipps K (2009) Surgical management of supratentorial ependymomas. Childs Nerv Syst 25:1215–1220

    Article  PubMed  Google Scholar 

  37. Armstrong GT, Liu Q, Yasui Y, Huang S, Ness KK, Leisenring W et al (2009) Long-term outcomes among adult survivors of childhood central nervous system malignancies in the Childhood Cancer Survivor Study. J Natl Cancer Inst 101:946–958

    Article  PubMed  Google Scholar 

  38. Merchant TE, Li C, Xiong X, Kun LE, Boop FA, Sanford RA (2009) Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol 10:258–266

    Article  PubMed  Google Scholar 

  39. Tabori U, Ma J, Carter M, Zielenska M, Rutka J, Bouffet E et al (2006) Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma. J Clin Oncol 24:1522–1528

    Article  PubMed  CAS  Google Scholar 

  40. Ridley L, Rahman R, Brundler MA, Ellison D, Lowe J, Robson K et al (2008) Multifactorial analysis of predictors of outcome in pediatric intracranial ependymoma. Neuro Oncol 10:675–689

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

This research was supported in part by NIH-PO1 NS-42927-27A2 (DHK, MB); NIH Brain Tumor SPORE grant P50 CA097257 (DHK, MYP, MSB, MDP), Nancy and Stephen Grand Philanthropic Fund (DHK), The V Foundation (DHK), and The Thrasher Foundation (DHK).

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Authors and Affiliations

  1. Departments of Radiation Oncology, Neurosurgery, and Pediatrics, University of California, San Francisco and UCSF Benioff Children’s Hospital, 1600 Divisadero Street, Suite H1031, San Francisco, CA, 94115-1708, USA

    Sunanda Pejavar, Mei-Yin Polley, Sarah Rosenberg-Wohl, Sravana Chennupati, Michael D. Prados, Mitchel S. Berger, Anuradha Banerjee, Nalin Gupta & Daphne Haas-Kogan

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  1. Sunanda Pejavar
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  2. Mei-Yin Polley
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Correspondence to Daphne Haas-Kogan.

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Pejavar, S., Polley, MY., Rosenberg-Wohl, S. et al. Pediatric intracranial ependymoma: the roles of surgery, radiation and chemotherapy. J Neurooncol 106, 367–375 (2012). https://doi.org/10.1007/s11060-011-0671-9

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  • DOI: https://doi.org/10.1007/s11060-011-0671-9

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