Evidence for involvement of a tumor suppressor gene on 1p in malignant peripheral nerve sheath tumors.
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.
Van Roy
Nadine
N
Department of Medical Genetics, Ghent University Hospital, Ghent, Belgium.
Van Gele
Mireille
M
Vandesompele
Jo
J
Messiaen
Ludwine
L
Van Belle
Simon
S
Sciot
Raf
R
Mortéle
Koen
K
Gyselinck
Jan
J
Michiels
Erna
E
Forsyth
Ramses
R
Van Marck
Erik
E
De Paepe
Anne
A
Speleman
Frank
F
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*