Atypical cytogenetic aberrations in two childhood peripheral primitive neuroectodermal Tumors
✍ Scribed by Dr. Jeffrey C. Murray; Claire Langston; Zoann E. Dreyer; Charles T. Stephenson; William J. Pokorny; Marc E. Horowitz; Linda D. Cooley
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 810 KB
- Volume
- 12
- Category
- Article
- ISSN
- 1045-2257
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✦ Synopsis
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.