title: Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature.
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.
authors.affiliation: Division of Pediatric Neurosurgery, Rainbow Babies and Children's Hospital, Cleveland, Ohio 44106, USA.
authors.forename: Sunil
authors.initials: S
authors.lastname: Manjila
authors.forename: Abhishek
authors.initials: A
authors.lastname: Ray
authors.forename: Yin
authors.initials: Y
authors.lastname: Hu
authors.forename: Dan X
authors.initials: DX
authors.lastname: Cai
authors.forename: Mark L
authors.initials: ML
authors.lastname: Cohen
authors.forename: Alan R
authors.initials: AR
authors.lastname: Cohen
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000706:statistics & numerical data; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000145:classification / Q000473:pathology* / Q000601:surgery; D009447:Neuroblastoma / Q000145:classification / Q000473:pathology / Q000601:surgery; D018242:Neuroectodermal Tumors, Primitive / Q000145:classification / Q000473:pathology* / Q000601:surgery; D019581:Neuropil / Q000473:pathology*; D014944:World Health Organization