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Molecular and gene array analyses of rare pediatric mesenchymal tumors: malignant intracranial ectomesenchymoma compared to rhabdomyosarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma
Author(s) -
KleinschmidtDeMasters Bette K.,
Lovell Mark A.,
Donson Andrew M,
Wilkinson C Corbett,
AddoYobo Steven O,
Lillehei Kevin O,
Foreman Nicholas K
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a27-c
Subject(s) - rhabdomyosarcoma , sarcoma , malignant peripheral nerve sheath tumor , ewing's sarcoma , pathology , neuroblastoma , medicine , cancer research , biology , immunohistochemistry , cell culture , genetics
Malignant ectomesenchymoma (EM) is a pediatric sarcoma containing mature ganglion cells and rhabdomyosarcoma (RMS); less than a half‐dozen intracranial examples have been reported. Limited literature suggests they may be related to either RMS or Ewing sarcoma (EW). An intracranial EM was investigated and results compared with RMS and EW, as well as malignant peripheral nerve sheath tumor (MPNST). Cytogenetic studies showed no overlap between EM and MPNST. RT‐PCR testing for the classic gene rearrangements seen in RMS [PAX3/FKHR] and Ewing's sarcoma (EWS/FLI‐1 & EWS/ERG) were negative in the EM. Affymetrix gene array analyses on RMS (n=8), EW (n=2), and MPNST (n=2) showed tight clustering of the EM with the MPNSTs, but divergence from all RMS and EW, as well as from other types of pediatric brain tumors (n=52). The overlap between EM and MPNST suggests a common stem cell origin/or embryonic gene recapitulation for these tumors and provides novel insights into their underlying biology.