Article
Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients.
Neurobiology of disease - 1 Jun 2004
Frahm Silke, Mautner Victor-F, Brems Hilde, Legius Eric, Debiec-Rychter Maria, Friedrich Reinhard E, Knöfel Wolfram T, Peiper Matthias, Kluwe Lan
Abstract excerpt
Neurofibromatosis type 1 (NF1) patients have an 8-13% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNST) which have a very poor prognosis. In this study, cells from eight MPNSTs (six primary and two recurrences) of six clinically and genetically well-characterized NF1 patients were taken into culture. Tracing of loss of heterozygosity (LOH) of the NF1, p53, and p16 gene regions or of...
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