Absence of p53 mutations in childhood central nervous system primitive neuroectodermal tumors.
The primitive neuroectodermal tumor of the central nervous system is one of a number of tumors in which deletions on chromosome 17p have been identified. The tumor suppressor gene, p53, is located in the region of the deletion. To determine if the p53 gene is involved in the development of primitive neuroectodermal tumors, deoxyribonucleic acid (DNA) blot analysis, ribonucleic acid blot analysis, and p53 complementary DNA sequencing were performed on 34 primitive neuroectodermal tumors removed from children. No rearrangement in the gene was detected in 21 tumors. The p53 messenger ribonucleic acid was of the expected size in all 18 tumors for which ribonucleic acid was available. Sequencing of p53 Exons 5 through 9 revealed a mutation in the cell line DAOY and in only 1 of 14 tumors examined. A DNA rearrangement was detected in the DNA from one tumor with a probe mapping to the distal portion of 17p. Taken together, these data suggest that the p53 gene is not involved in the development of most primitive neuroectodermal tumors. In addition, a gene of interest may be present on distal 17p.
Division of Neurosurgery, Childrens Hospital, Los Angeles, California.
C
C
Raffel
G A
GA
Thomas
D M
DM
Tishler
S
S
Lassoff
J C
JC
Allen
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000235:genetics; D002885:Chromosomes, Human, Pair 16; D003131:Combined Modality Therapy; D015342:DNA Probes; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009373:Neoplasms, Germ Cell and Embryonal / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D016133:Polymerase Chain Reaction; D013116:Spinal Cord / Q000473:pathology; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
D015342:DNA Probes; D016159:Tumor Suppressor Protein p53