TY - JOUR
T1 - Embryonal tumors with abundant neuropil and true Rosettes
T2 - A distinctive CNS primitive neuroectodermal tumor
AU - Gessi, Marco
AU - Giangaspero, Felice
AU - Lauriola, Libero
AU - Gardiman, Marina
AU - Scheithauer, Bernd W.
AU - Halliday, William
AU - Hawkins, Cynthia
AU - Rosenblum, Marc K.
AU - Burger, Peter C.
AU - Eberhart, Charles G.
PY - 2009/2
Y1 - 2009/2
N2 - Embryonal neoplasms of the central nervous system (CNS) generally arise in the early years of life and behave in a clinically aggressive manner, but vary somewhat in their microscopic appearance. Several groups have reported examples of an embryonal tumor with combined histologic features of ependymoblastoma and neuroblastoma, a lesion referred to as "embryonal tumor with abundant neuropil and true rosettes" (ETANTR). Herein, we present 22 new cases, and additional clinical follow-up on our 7 initially reported cases, to better define the histologic features and clinical behavior of this distinctive neoplasm. It affects infants and arises most often in cerebral cortex, the cerebellum and brainstem being less frequent sites. Unlike other embryonal tumors of the CNS, girls are more commonly affected than boys. On neuroimaging, the tumors appear as large, demarcated, solid masses featuring patchy or no contrast enhancement. Five of our cases (18%) were at least partly cystic. Distinctive microscopic features include a prominent background of mature neuropil punctuated by true rosettes formed of pseudo-stratified embryonal cells circumferentially disposed about a central lumen (true rosettes). Of the 25 cases with available follow-up, 19 patients have died, their median survival being 9 months. Performed on 2 cases, cytogenetic analysis revealed extra copies of chromosome 2 in both. We believe that the ETANTR represents a histologically distinctive form of CNS embryonal tumor.
AB - Embryonal neoplasms of the central nervous system (CNS) generally arise in the early years of life and behave in a clinically aggressive manner, but vary somewhat in their microscopic appearance. Several groups have reported examples of an embryonal tumor with combined histologic features of ependymoblastoma and neuroblastoma, a lesion referred to as "embryonal tumor with abundant neuropil and true rosettes" (ETANTR). Herein, we present 22 new cases, and additional clinical follow-up on our 7 initially reported cases, to better define the histologic features and clinical behavior of this distinctive neoplasm. It affects infants and arises most often in cerebral cortex, the cerebellum and brainstem being less frequent sites. Unlike other embryonal tumors of the CNS, girls are more commonly affected than boys. On neuroimaging, the tumors appear as large, demarcated, solid masses featuring patchy or no contrast enhancement. Five of our cases (18%) were at least partly cystic. Distinctive microscopic features include a prominent background of mature neuropil punctuated by true rosettes formed of pseudo-stratified embryonal cells circumferentially disposed about a central lumen (true rosettes). Of the 25 cases with available follow-up, 19 patients have died, their median survival being 9 months. Performed on 2 cases, cytogenetic analysis revealed extra copies of chromosome 2 in both. We believe that the ETANTR represents a histologically distinctive form of CNS embryonal tumor.
KW - Divergent differentiation
KW - Embryonal tumor
KW - Ependymoblastic rosettes
KW - Neuropil
KW - Pediatric brain tumors
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U2 - 10.1097/PAS.0b013e318186235b
DO - 10.1097/PAS.0b013e318186235b
M3 - Article
C2 - 18987548
AN - SCOPUS:60249103002
SN - 0147-5185
VL - 33
SP - 211
EP - 217
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 2
ER -