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 C11orf–RelA 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.
This is a preview of subscription content, log in to check access
This work is dedicated to the memory of Professor Nobutaka Kawahara.
Louis DN, Ohgaki H, Wiestler OD et al (2016) WHO classification of tumours of the central nervous system. IARC Publications, LyonGoogle Scholar
Nazar GB, Hoffman HJ, Becker LE et al (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72:408–417CrossRefPubMedGoogle Scholar
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
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
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
Massimino M, Solero CL, Garre ML et al (2011) Second-look surgery for ependymoma: the Italian experience. J Neurosurg Pediatr 8:246–250CrossRefPubMedGoogle Scholar
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
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
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
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
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