PMID- 17332950
OWN - NLM
STAT- MEDLINE
DCOM- 20070927
LR  - 20220716
IS  - 0167-594X (Print)
IS  - 0167-594X (Linking)
VI  - 84
IP  - 1
DP  - 2007 Aug
TI  - Embryonal tumor with abundant neuropil and true rosettes (ETANTR): report of a 
      case with prominent neurocytic differentiation.
PG  - 91-8
AB  - We report a case of a 2 year-old boy who initially presented with macrocephaly 
      and severe global developmental delay. Imaging revealed a large left 
      temporo-parietal mass that was lobulated, calcified, focally enhancing and 
      partially cystic. A second surgery was required for tumor recurrence 
      approximately one year later, and tissue from that resection proved to be 
      diagnostic for an embryonal tumor with abundant neuropil and true rosettes 
      (ETANTR). Only 12 cases of this rare pediatric embryonal tumor have been 
      previously documented, and as of 2000, the WHO has not recognized ETANTR as a 
      distinct entity (Kleihues P, Cavenee WK (2000) International agency for research 
      on cancer: pathology and genetics of tumors of the nervous system. IARC Press, 
      Lyon). As opposed to prior cases, our patient's tumor exhibited extensive 
      neurocytic elements. Two recently described cases were examined via fluorescence 
      in situ hybridization (FISH), with one demonstrating isochromosome 17q (i17q) and 
      the second exhibiting polysomies of chromosomes 2, 8, 17 and 22 (Fuller C, 
      Fouladi M, Gajjar A, Dalton J, Sanford RA, Helton KJ (2000) Am J Clin Pathol 126: 
      277-283). Via FISH analysis, we found normal dosages of chromosomes 2, 8 and 17. 
      Our case expands the histopathologic spectrum of ETANTR, illustrating marked 
      neuronal differentiation towards neurocytes. The lack of common PNET-associated 
      FISH abnormalities in this case adds to the limited cytogenetic genetic data on 
      this rare pediatric embryonal neoplasm.
FAU - Dunham, Christopher
AU  - Dunham C
AD  - Division of Neuropathology, Department of Pathology and Immunology, Washington 
      University School of Medicine, 660 S. Euclid, Campus Box, 8118, St. Louis, MO 
      63110, USA.
FAU - Sugo, Ella
AU  - Sugo E
FAU - Tobias, Vivienne
AU  - Tobias V
FAU - Wills, Edward
AU  - Wills E
FAU - Perry, Arie
AU  - Perry A
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20070228
PL  - United States
TA  - J Neurooncol
JT  - Journal of neuro-oncology
JID - 8309335
SB  - IM
MH  - Brain Neoplasms/*pathology/surgery/therapy
MH  - Child, Preschool
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Neoplasm Recurrence, Local/*parasitology/surgery/therapy
MH  - Neuroectodermal Tumors, Primitive/*pathology/surgery/therapy
MH  - Neurons/pathology
MH  - Neuropil/*pathology
MH  - Rosette Formation
EDAT- 2007/03/03 09:00
MHDA- 2007/09/28 09:00
CRDT- 2007/03/03 09:00
PHST- 2006/10/30 00:00 [received]
PHST- 2007/01/29 00:00 [accepted]
PHST- 2007/03/03 09:00 [pubmed]
PHST- 2007/09/28 09:00 [medline]
PHST- 2007/03/03 09:00 [entrez]
AID - 10.1007/s11060-007-9346-y [doi]
PST - ppublish
SO  - J Neurooncol. 2007 Aug;84(1):91-8. doi: 10.1007/s11060-007-9346-y. Epub 2007 Feb 
      28.