title: Evidence for involvement of a tumor suppressor gene on 1p in malignant peripheral nerve sheath tumors.
abstract: Malignant peripheral nerve sheath tumors (MPNST) are rare soft-tissue malignancies. The genetic basis of these tumors is still poorly understood. Cytogenetic analyses predominantly revealed complex karyotypes, precluding the identification of recurrent chromosomal changes. We report loss of 1p material in a near-diploid karyotype with few or no additional structural chromosome changes in two sporadic cases of MPNST, indicating an important role of 1p loss in MPNST development. In one of these two tumors, a distal 1p deletion (1p31.2 approximately pter) was detected suggesting involvement of a tumor suppressor gene located within this distal region of 1p. Further evidence for recurrent 1p loss in MPNST was obtained by interphase fluorescence in situ hybridization, which showed loss of 1p material in 3 out of 13 tumors. These findings together with data from the literature suggest that loss of a tumor suppressor gene located within distal 1p is implicated in the pathogenesis of MPNST.
authors.affiliation: Department of Medical Genetics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Nadine
authors.initials: N
authors.lastname: Van Roy
authors.forename: Mireille
authors.initials: M
authors.lastname: Van Gele
authors.forename: Jo
authors.initials: J
authors.lastname: Vandesompele
authors.forename: Ludwine
authors.initials: L
authors.lastname: Messiaen
authors.forename: Simon
authors.initials: S
authors.lastname: Van Belle
authors.forename: Raf
authors.initials: R
authors.lastname: Sciot
authors.forename: Koen
authors.initials: K
authors.lastname: Mortéle
authors.forename: Jan
authors.initials: J
authors.lastname: Gyselinck
authors.forename: Erna
authors.initials: E
authors.lastname: Michiels
authors.forename: Ramses
authors.initials: R
authors.lastname: Forsyth
authors.forename: Erik
authors.initials: E
authors.lastname: Van Marck
authors.forename: Anne
authors.initials: A
authors.lastname: De Paepe
authors.forename: Frank
authors.initials: F
authors.lastname: Speleman
mesh_terms: D002648:Child; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics*; D005260:Female; D016147:Genes, Tumor Suppressor; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D019656:Loss of Heterozygosity / Q000235:genetics; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000235:genetics*