Embryonal Tumor with Multilayered Rosettes in a 3-Year-Old Girl: Case Report

Özet

Embryonal tumor with multilayered rosettes (ETMR) is a new entity in the group of primitive neuroectodermal tumors of the centralnervous system (CNS). It combines histopathological features of neuroblastoma and ependymoblastoma. This tumor occurs mostlyin the first five years of life. A hallmark cytogenetic abnormality at the chromosome locus 19q13.42 has been identified. Theauthors report a case of ETMR in the parietooccipital region in a 3-year-old girl who presented with features of raised intracranialtension. The exact diagnosis could be made by the finding of true rosettes in a single low power field. ETMR is a newly describedrare pediatric CNS tumor and large case studies regarding its differentiation from usual PNETs are lacking. Reporting each casewith the morphological features of this tumor would add to the existing data on these tumors as regards pathology, diagnosis andmanagement.