Loss of NF1 alleles distinguish sporadic from NF1-associated pilocytic astrocytomas
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Loss of NF1 alleles distinguish sporadic from NF1-associated pilocytic astrocytomas. / Kluwe, L; Hagel, C; Tatagiba, M; Thomas, S; Stavrou, D; Ostertag, H; von Deimling, A; Mautner, V F.
in: J NEUROPATH EXP NEUR, Jahrgang 60, Nr. 9, 01.09.2001, S. 917-20.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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T1 - Loss of NF1 alleles distinguish sporadic from NF1-associated pilocytic astrocytomas
AU - Kluwe, L
AU - Hagel, C
AU - Tatagiba, M
AU - Thomas, S
AU - Stavrou, D
AU - Ostertag, H
AU - von Deimling, A
AU - Mautner, V F
PY - 2001/9/1
Y1 - 2001/9/1
N2 - Pilocytic astrocytomas classified as WHO grade I typically arise in childhood and upon complete surgical removal carry a favorable prognosis. Children with neurofibromatosis 1 (NF1) have a vastly increased risk for pilocytic astrocytomas, especially for those of the optic nerve. Using 4 intragenic NF1 microsatellite markers, we examined losses of NF1 alleles on the long arm of chromosome 17 in 12 NF1-associated and 25 sporadic pilocytic astrocytomas. The TP53 gene region on the short arm of chromosome 17 was also examined in these tumors using 3 markers. Loss of 1 NF1 allele was detected in 11 of 12 (92%) informative NF1-associated pilocytic astrocytomas. In contrast, only 1 of 24 informative (4%) sporadic pilocytic astrocytomas exhibited allelic loss in the NF1 region. Among the 11 NF1-associated tumors with NF1 loss, 5 had also lost alleles on 17p. The high rate of NF1 allele loss in NF1-associated pilocytic astrocytomas suggests a tumor initiating or promoting action of the NF1 gene in these patients. On the other hand, the much lower rate of NF1-allele loss in sporadic pilocytic astrocytomas argues for only minor importance of NF1 in that patient group. The present data support different mechanisms in the formation of NF1-associated and sporadic pilocytic astrocytomas.
AB - Pilocytic astrocytomas classified as WHO grade I typically arise in childhood and upon complete surgical removal carry a favorable prognosis. Children with neurofibromatosis 1 (NF1) have a vastly increased risk for pilocytic astrocytomas, especially for those of the optic nerve. Using 4 intragenic NF1 microsatellite markers, we examined losses of NF1 alleles on the long arm of chromosome 17 in 12 NF1-associated and 25 sporadic pilocytic astrocytomas. The TP53 gene region on the short arm of chromosome 17 was also examined in these tumors using 3 markers. Loss of 1 NF1 allele was detected in 11 of 12 (92%) informative NF1-associated pilocytic astrocytomas. In contrast, only 1 of 24 informative (4%) sporadic pilocytic astrocytomas exhibited allelic loss in the NF1 region. Among the 11 NF1-associated tumors with NF1 loss, 5 had also lost alleles on 17p. The high rate of NF1 allele loss in NF1-associated pilocytic astrocytomas suggests a tumor initiating or promoting action of the NF1 gene in these patients. On the other hand, the much lower rate of NF1-allele loss in sporadic pilocytic astrocytomas argues for only minor importance of NF1 in that patient group. The present data support different mechanisms in the formation of NF1-associated and sporadic pilocytic astrocytomas.
KW - Adolescent
KW - Adult
KW - Alleles
KW - Astrocytoma
KW - Brain Neoplasms
KW - Cerebellum
KW - Chromosomes, Human, Pair 17
KW - Female
KW - Humans
KW - Loss of Heterozygosity
KW - Male
KW - Microsatellite Repeats
KW - Nerve Tissue Proteins
KW - Neurofibromin 1
KW - Optic Nerve
KW - Spinal Cord
KW - Temporal Lobe
M3 - SCORING: Journal article
C2 - 11556548
VL - 60
SP - 917
EP - 920
JO - J NEUROPATH EXP NEUR
JF - J NEUROPATH EXP NEUR
SN - 0022-3069
IS - 9
ER -