title: Atypical cytogenic aberrations in two childhood peripheral primitive neuroectodermal tumors.
abstract: Atypical cytogenetic abnormalities were detected in peripheral primitive neuroectodermal tumors (PPNET) of the extremity in two children. One had an osseous tumor with a balanced reciprocal translocation, t(5;9)(q22;q32), and had a complete response to therapy. The other had a non-osseous tumor with an interstitial deletion, del(18)(q12.2q21.2), was resistant to combination therapy, and at autopsy had evidence of possible clonal evolution with the karyotype 46,XX der(8)t(8;8)(p11.2;q13), inv(16)(p13.2q12),del(18)(q12.2q21.2). Neither tumor demonstrated the t(11;22)(q24;q12) typically found in Ewing's sarcoma and PPNET, suggesting heterogeneity of the cytogenetic aberrations seen in this rare childhood malignancy.
authors.affiliation: Section of Hematology-Oncology, Texas Children's Cancer Center, Houston.
authors.forename: J C
authors.initials: JC
authors.lastname: Murray
authors.forename: C
authors.initials: C
authors.lastname: Langston
authors.forename: Z E
authors.initials: ZE
authors.lastname: Dreyer
authors.forename: C T
authors.initials: CT
authors.lastname: Stephenson
authors.forename: W J
authors.initials: WJ
authors.lastname: Pokorny
authors.forename: M E
authors.initials: ME
authors.lastname: Horowitz
authors.forename: L D
authors.initials: LD
authors.lastname: Cooley
mesh_terms: D001859:Bone Neoplasms / Q000235:genetics*; D002648:Child; D002869:Chromosome Aberrations; D005260:Female; D006801:Humans; D007621:Karyotyping; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics*; D012983:Soft Tissue Neoplasms / Q000235:genetics*