Advertisement

Brain Tumor Pathology

, Volume 34, Issue 4, pp 160–164 | Cite as

Encouraging option of multi-staged gross total resection for a C11orf-RelA fusion-positive supratentorial anaplastic ependymoma

  • Taishi Nakamura
  • Kohei Fukuoka
  • Junji Ikeda
  • Masahiro Yoshitomi
  • Naoko Udaka
  • Reo Tanoshima
  • Kensuke Tateishi
  • Shoji Yamanaka
  • Koichi Ichimura
  • Tetsuya Yamamoto
Case Report

Abstract

Ependymomas are primary neuroepithelial malignancies that mainly occur during childhood, and arise from ependymal cells along the ventricular systems of the CNS. Recently, it was elucidated that two-thirds of supratentorial (ST) ependymomas harbor oncogenic fusions of RELA, whose protein product is the principal effector of canonical NF-κB signaling. RELA fusion proteins activate signaling for tumor proliferation and malignant progression, resulting in poorer prognoses in these patients compared to those in patients with other ST ependymomas. In this study, we encountered a case of C11orfRelA fusion-positive ST anaplastic ependymoma that was diagnosed in first tumor resection surgery of multi-staged gross total resection with molecular evidence. In ependymomas, regardless of tumor location or pathological grade, subtotal resection is associated with higher rates of mortality compared with GTR. Molecular analysis based on the application of recent molecular knowledge for ST ependymomas performs a role in appropriate and individualized treatment strategies.

Keywords

Ependymoma RelA Supratentrial 

Notes

Acknowledgements

This work is dedicated to the memory of Professor Nobutaka Kawahara.

References

  1. 1.
    Louis DN, Ohgaki H, Wiestler OD et al (2016) WHO classification of tumours of the central nervous system. IARC Publications, LyonGoogle Scholar
  2. 2.
    Nazar GB, Hoffman HJ, Becker LE et al (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417CrossRefPubMedGoogle Scholar
  3. 3.
    Parker M, Mohankumar KM, Punchihewa C et al (2014) C11orf95–RELA fusions drive oncogenic NF-kappaB signalling in ependymoma. Nature 506:451–455CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Pietsch T, Wohlers I, Goschzik T et al (2014) Supratentorial ependymomas of childhood carry C11orf95–RELA fusions leading to pathological activation of the NF-kappaB signaling pathway. Acta Neuropathol 127:609–611CrossRefPubMedGoogle Scholar
  5. 5.
    Mack SC, Witt H, Piro RM et al (2014) Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature 506:445–450CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Pajtler KW, Witt H, Sill M et al (2015) Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 27:728–743CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Sturm D, Orr BA, Toprak UH et al (2016) New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell 164:1060–1072CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Merchant TE, Li C, Xiong X et al (2009) Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol 10:258–266CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Cage TA, Clark AJ, Aranda D et al (2013) A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr 11:673–681CrossRefPubMedGoogle Scholar
  10. 10.
    Massimino M, Solero CL, Garre ML et al (2011) Second-look surgery for ependymoma: the Italian experience. J Neurosurg Pediatr 8:246–250CrossRefPubMedGoogle Scholar
  11. 11.
    Cartmill M, Hewitt M, Walker D et al (2001) The use of chemotherapy to facilitate surgical resection in pleomorphic xanthoastrocytoma: experience in a single case. Child’s Nerv Syst 17:563–566CrossRefGoogle Scholar
  12. 12.
    Valera ET, Serafini LN, Machado HR, Tone LG (2003) Complete surgical resection in children with low-grade astrocytomas after neoadjuvant chemotherapy. Child’s Nerv Syst 19:86–90Google Scholar
  13. 13.
    Valera ET, Machado HR, Santos AC et al (2005) The use of neoadjuvant chemotherapy to achieve complete surgical resection in recurring supratentorial anaplastic ependymoma. Child’s Nerv Syst 21:230–233CrossRefGoogle Scholar
  14. 14.
    Huang J, Barbera L, Brouwers M et al (2003) Does delay in starting treatment affect the outcomes of radiotherapy? A systematic review. J Clin Oncol 21:555–563CrossRefPubMedGoogle Scholar
  15. 15.
    van den Bent MJ, Afra D, de Witte O et al (2005) Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Lancet 366:985–990CrossRefPubMedGoogle Scholar

Copyright information

© The Japan Society of Brain Tumor Pathology 2017

Authors and Affiliations

  • Taishi Nakamura
    • 1
  • Kohei Fukuoka
    • 2
  • Junji Ikeda
    • 3
  • Masahiro Yoshitomi
    • 3
  • Naoko Udaka
    • 4
  • Reo Tanoshima
    • 3
  • Kensuke Tateishi
    • 1
  • Shoji Yamanaka
    • 4
  • Koichi Ichimura
    • 2
  • Tetsuya Yamamoto
    • 1
  1. 1.Department of Neurosurgery, Graduate School of MedicineYokohama City UniversityYokohamaJapan
  2. 2.Division of Brain Tumor Translational ResearchNational Cancer Center Research InstituteTokyoJapan
  3. 3.Department of Pediatrics, Graduate School of MedicineYokohama City UniversityYokohamaJapan
  4. 4.Department of Pathology, Graduate School of MedicineYokohama City UniversityYokohamaJapan

Personalised recommendations