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Atypical cytogenetic aberrations in two childhood peripheral primitive neuroectodermal Tumors
Author(s) -
Murray Jeffrey C.,
Langston Claire,
Dreyer Zoann E.,
Stephenson Charles T.,
Pokorny William J.,
Horowitz Marc E.,
Cooley Linda D.
Publication year - 1995
Publication title -
genes, chromosomes and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.754
H-Index - 119
eISSN - 1098-2264
pISSN - 1045-2257
DOI - 10.1002/gcc.2870120209
Subject(s) - chromosomal translocation , sarcoma , karyotype , pathology , malignancy , cytogenetics , biology , chromosome , medicine , genetics , gene
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.