Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*.
Ependymoblastoma is a diagnostic label that has been applied to a variety of rare central nervous system (CNS) tumors over the last eight decades. Consequently, there is uncertainty about whether such an entity exists and what its characteristic features might be. The current study, based on 14 cases from our institutional archives and identified by the search terms "ependymoblastoma,"ependymoblastomatous," "ependymoblastic" or "PNET with ependymal differentiation," aimed to test the hypothesis that the ependymoblastoma is a distinct and recognizable entity. Ependymoblastic rosettes are a key diagnostic feature and were present in 11/14 (79%) tumors, eight (73%) of which were embryonal tumors with abundant areas of neuropil-like differentiation. Three other cases showed rare ependymoblastic rosettes in the histopathological setting of a typical primitive neuroectodermal tumor (PNET), medulloblastoma (MB) or atypical teratoid/rhabdoid tumor (AT/RT). The remaining cases were all embryonal tumors with structures that mimicked ependymoblastic rosettes. Our results indicate that ependymoblastic rosettes are most frequently encountered in embryonal tumors with abundant neuropil and less frequently in other CNS embryonal neoplasms, including PNET, MB and AT/RT. We believe that ependymoblastoma as a diagnosis is neither precise nor specific and that it is time once and for all to retire this diagnosis from the lexicon of neuropathology.
Judkins
Alexander R
AR
Division of Neuropathology, Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Ellison
David W
DW
D002454:Cell Differentiation / Q000502:physiology; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005724:Ganglia / Q000166:cytology; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D008527:Medulloblastoma / Q000473:pathology; D009373:Neoplasms, Germ Cell and Embryonal / Q000473:pathology; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology*; D009474:Neurons / Q000502:physiology; D019581:Neuropil / Q000473:pathology; D018335:Rhabdoid Tumor / Q000473:pathology; D012397:Rosette Formation; D012737:Sex Factors; D013724:Teratoma / Q000473:pathology; D009626:Terminology as Topic