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| Gain of chromosome arm 17q and adverse outcome in patients with neuroblastoma |
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| Author(s): Bown N, Cotterill S, Lastowska M, O'Neill S, Pearson ADJ, Plantaz D, Meddeb M, Danglot G, Brinkschmidt C, Christiansen H, Laureys G, Speleman F |
| Source: NEW ENGLAND JOURNAL OF MEDICINE Volume: 340 Issue: 25 Pages: 1954-1961 Published: JUN 24 1999 |
| Times Cited: 201 References: 29 |
| Abstract: Background Gain of genetic material from chromosome arm 17q (gain of segment 17q21-qter) is the most frequent cytogenetic abnormality of neuroblastoma cells. This gain has been associated with advanced disease, patients who are greater than or equal to 1 year old, deletion of chromosome arm 1p, and amplification of the N-myc oncogene, all of which predict an adverse outcome. We investigated these associations and evaluated the prognostic importance of the status of chromosome 17. Methods We compiled molecular cytogenetic analyses of chromosome 17 in primary neuroblastomas in 313 patients at six European centers. Clinical and survival information were collected, along with data on 1p, N-myc, and ploidy.
Results Unbalanced gain of segment 17q21-qter was found in 53.7 percent of the tumors, whereas the chromosome was normal in 46.3 percent. The gain of 17q was characteristic of advanced tumors and of tumors in children greater than or equal to 1 year of age and was strongly associated with the deletion of 1p and amplification of N-myc. No tumor showed amplification of N-myc in the absence of either deletion of 1p or gain of 17q. Gain of 17q was a significant predictive factor for adverse outcome in univariate analysis. Among the patients with this abnormality, overall survival at five years was 30.6 percent (95 percent confidence interval, 21 to 40 percent), as compared with 86.0 percent (95 percent confidence interval, 78 to 91 percent) among those with normal 17q status. In multivariate analysis, gain of 17q was the most powerful prognostic factor, followed by the presence of stage 4 disease and deletion of 1p (hazard ratios, 3.4, 2.3, and 1.9, respectively).
Conclusions Gain of chromosome segment 17q21-qter is an important prognostic factor in children with neuroblastoma. (N Engl J Med 1999;340:1954-61.) (C) 1999, Massachusetts Medical Society.
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| Document Type: Article |
| Language: English |
| Reprint Address: Bown, N (reprint author), Univ Newcastle Upon Tyne, Dept Human Genet, 19-20 Claremont Pl, Newcastle Upon Tyne NE2 4AA, Tyne & Wear England |
Addresses:
1. Univ Newcastle Upon Tyne, Dept Human Genet, Newcastle Upon Tyne NE2 4AA, Tyne & Wear England 2. Univ Newcastle Upon Tyne, Inst Child Hlth, Newcastle Upon Tyne NE2 4AA, Tyne & Wear England 3. Univ Hosp Ctr, Dept Pediat, Grenoble, France 4. Inst Gustave Roussy, Villejuif, France 5. Univ Munster, Gerhard Domagk Inst Pathol, D-4400 Munster, Germany 6. Univ Marburg, Kinderklin, Dept Pediat Hematol & Oncol, Marburg, Germany 7. State Univ Ghent Hosp, Dept Pediat Oncol, B-9000 Ghent, Belgium 8. State Univ Ghent Hosp, Ctr Med Genet, B-9000 Ghent, Belgium |
| Publisher: MASSACHUSETTS MEDICAL SOC, WALTHAM WOODS CENTER, 860 WINTER ST,, WALTHAM, MA 02451-1413 USA |
| Subject Category: Medicine, General & Internal |
| IDS Number: 210CX |
| ISSN: 0028-4793 |
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