title: Unusual genetic aberrations including a deletion of KLF6 tumor suppressor gene revealed by integrated cytogenetic approaches in a pediatric ewing sarcoma.
abstract: Ewing sarcoma is the third most common sarcoma in children and young adults. Its characteristic chromosomal rearrangement results in a chimerical EWSR1-ETS transcription factor. Secondary genetic alterations are very common. Membranous expression of CD99 is seen in almost all tumors. We report 2 unusual cytogenetic findings in a pediatric Ewing sarcoma, an insertion of the MIC2 gene encoding CD99 from Xp to 10p and a submicroscopic deletion of the well-known tumor supressor gene KLF6. The latter has not been described previously in pediatric neoplasms. Molecular pathways in tumorigenesis and genetic complexity in cancer are discussed.
authors: *Department of Pediatrics, Section of Oncology †Department of Pathology and Laboratory Medicine, Drexel University College of Medicine and St. Christopher's Hospital for Children, Philadelphia, PA.
authors: Jason C
authors: JC
authors: White
authors: Gregory E
authors: GE
authors: Halligan
authors: Jean-Pierre
authors: JP
authors: de Chadarévian
authors: Judy M
authors: JM
authors: Pascasio
authors: Hope H
authors: HH
authors: Punnet
authors: Jinglan
authors: J
authors: Liu
mesh_terms: D000071443:12E7 Antigen; D015703:Antigens, CD / Q000235:genetics; D001859:Bone Neoplasms / Q000235:genetics*; D015815:Cell Adhesion Molecules / Q000235:genetics; D002648:Child; D017353:Gene Deletion; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D000075723:Kruppel-Like Factor 6; D051741:Kruppel-Like Transcription Factors / Q000235:genetics*; D008297:Male; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000235:genetics*
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; C105465:KLF6 protein, human; D000075723:Kruppel-Like Factor 6; D051741:Kruppel-Like Transcription Factors; D011518:Proto-Oncogene Proteins