Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature

Sunil Manjila, Abhishek Ray, Yin Hu, Dan X. Cai, Mark L. Cohen, Alan R. Cohen

Research output: Contribution to journalArticlepeer-review

Abstract

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently identified variant of primitive neuroectodermal tumor, with fewer than 50 cases reported in the literature to date. Histologically, this tumor has features of ependymoblastoma and neuroblastoma, demonstrating areas of fine fibrillary neuropil intermingled with ependymoblastic rosettes and zones of undifferentiated neuroepithelial cells. However, ETANTR is distinguished pathologically from other embryonal tumors by the striking abundance of neuropil. Clinically, ETANTRs have shown high malignant potential and poor clinical outcome despite aggressive treatment. The authors describe 2 illustrative surgical cases of ETANTR, one involving the longest reported survival in the literature to date. The other had a poor outcome despite high-dose adjuvant chemotherapy with sequential autologous hematopoietic stem cell rescue. The authors review the natural history and treatment strategies available for this unusual malignant pediatric brain tumor.

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalNeurosurgical focus
Volume30
Issue number1
DOIs
StatePublished - Jan 2011
Externally publishedYes

Keywords

  • Brain tumor
  • Embryonal tumor with abundant neuropil and true rosettes
  • Ependymoblastoma
  • Neuropil
  • Primitive neuroectodermal tumor
  • WHO classification

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature'. Together they form a unique fingerprint.

Cite this