TY - JOUR
T1 - Embryonal Tumor with Abundant Neuropil and True Rosettes
T2 - A Distinct Immunohistochemical Pattern
AU - Al-Salam, Suhail
AU - Al Alashari, Mouied
N1 - Publisher Copyright:
© 2016 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2016/7/1
Y1 - 2016/7/1
N2 - Embryonal tumors with abundant neuropil and true rosettes (ETANTR) are rare pediatric embryonal neoplasms that combine features of neuroblastoma and ependymoblastoma. We report a distinct immunohistochemical-staining pattern of ETANTR in a 12-month-old baby who presented with a supratentorial mass. The tumor exhibited a characteristic biphasic pattern of neuropil-rich areas and patchy cellular neuropil-poor areas. The neoplastic cells in neuropil-rich areas are diffusely immunoreactive to chromogranin A, synaptophysin, neurofilament, and CD56, but show no immunoreactivity to nestin, SOX2, WT-1, β-catenin, and vimentin. While the cells in neuropil-poor areas, including ependymoblastic and Flexner-Wintersteiner rosettes, are diffusely immunoreactive to nestin, SOX2, WT-1, β-catenin, and vimentin but show no immunoreactivity to chromogranin A, synaptophysin, neurofilament, and CD56. Ependymoblastic rosettes show luminal membranous immunoreactivity to EMA. We believe that ETANTR has a distinct histologic and immunohistochemical pattern supporting the embryonal origin of this tumor with divergent neuroblastic and primitive glial differentiation.
AB - Embryonal tumors with abundant neuropil and true rosettes (ETANTR) are rare pediatric embryonal neoplasms that combine features of neuroblastoma and ependymoblastoma. We report a distinct immunohistochemical-staining pattern of ETANTR in a 12-month-old baby who presented with a supratentorial mass. The tumor exhibited a characteristic biphasic pattern of neuropil-rich areas and patchy cellular neuropil-poor areas. The neoplastic cells in neuropil-rich areas are diffusely immunoreactive to chromogranin A, synaptophysin, neurofilament, and CD56, but show no immunoreactivity to nestin, SOX2, WT-1, β-catenin, and vimentin. While the cells in neuropil-poor areas, including ependymoblastic and Flexner-Wintersteiner rosettes, are diffusely immunoreactive to nestin, SOX2, WT-1, β-catenin, and vimentin but show no immunoreactivity to chromogranin A, synaptophysin, neurofilament, and CD56. Ependymoblastic rosettes show luminal membranous immunoreactivity to EMA. We believe that ETANTR has a distinct histologic and immunohistochemical pattern supporting the embryonal origin of this tumor with divergent neuroblastic and primitive glial differentiation.
KW - PNETs
KW - embryonal tumor with abundant neuropil and true rosettes
KW - pediatric CNS neoplasms
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U2 - 10.1097/PAI.0000000000000285
DO - 10.1097/PAI.0000000000000285
M3 - Article
C2 - 26658063
AN - SCOPUS:84949571164
SN - 1541-2016
VL - 24
SP - e41-e49
JO - Applied Immunohistochemistry and Molecular Morphology
JF - Applied Immunohistochemistry and Molecular Morphology
IS - 6
ER -