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  • Title: Atypical cytogenic aberrations in two childhood peripheral primitive neuroectodermal tumors.
    Author: Murray JC, Langston C, Dreyer ZE, Stephenson CT, Pokorny WJ, Horowitz ME, Cooley LD.
    Journal: Genes Chromosomes Cancer; 1995 Feb; 12(2):142-7. PubMed ID: 7535087.
    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.
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