Disseminated neuroblastomas under 1 year of age: cell biology and prognosis |
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Authors: | Lampert Fritz Christiansen Holger Berner Frank Terpe Hans-Joachim Berthold Frank |
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Institution: | (1) Department of Pediatrics, Hematology and Oncology, University of Giessen, Germany;(2) Department of Pathology, University of Giessen, Germany |
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Abstract: | Tumor specimens of 203 infants with neuroblastomas of different clinical stages — registered in successive multicenter clinical trials of the German Society of Pediatric Oncology — could be examined for N-myc amplification, chromosome 1-ploidy and — structure, CD44 std. expression (in tumor tissue, and also in patients sera).Eightyseven (= 43%) of these infants had a non-localized, disseminated neuroblastoma, mainly involving sympathetic nerve tissue, lymphnodes, liver, skin, bone marrow and bones (46 patients were classified into the 4s group, 41 patients in the true 4 group).If the clinical classification between stage 4 and stage 4swas neglected, then 17 of these infants (= 20%) had N-myc amplification (4—64 copies) with 16 already dead. Seven of 9 examined patients with true stage 4 had chromosome 1p aberrations (with N-myc amplification in 5), and among the dead there were 2 with CD44 negative expression.In another series, serum CD44 std. was measured by ELISA, and the highest (significantly different) Kruskal-Wallis mean rank values (147.8) were found in infants (n = 6) with stage 4s compared to the low mean-rank-value of 71.9 in patients with stage 4 (n = 65). Stage 1—3 patients (n = 42) had values of 99.8—88.6.Thus, infants with disseminated neuroblastomas, showing non-diploidy, normal chromosome 1p structure, non-N-myc amplification and high CD44 std. expression in tumor tissue, and also high CD44 std. values in serum, will have the highest chance of survival due to tumor-non-progression.On the other hand, N-myc amplification in the tumor cells was found to be characteristic for stage 4s neuroblastoma patients with tumor progression (n=6). Therefore, 4s neuroblastoma-patients with N-myc amplified tumors should be aggressively treated like true stage 4 tumor patients! |
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Keywords: | infantile disseminated neuroblastoma N-myc amplification differential prognosis |
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