TY - JOUR
T1 - Pediatric oligodendrogliomas
T2 - A study of molecular alterations on 1p and 19q using fluorescence in situ hybridization
AU - Raghavan, Ravi
AU - Balani, Jyoti
AU - Perry, Arie
AU - Margraf, Linda
AU - Vono, Mary B.
AU - Cai, Dan X.
AU - Wyatt, Robert E.
AU - Rushing, Elisabeth J.
AU - Bowers, Daniel C.
AU - Hynan, Linda S.
AU - White, Charles L.
PY - 2003/5/1
Y1 - 2003/5/1
N2 - Oligodendrogliomas (OGs) are rare in children and have not been well characterized from a molecular viewpoint. In adults, losses on chromosomes 1p and/or 19q are common in "oligodendroglial" neoplasms and are highly associated with chemosensitivity and greater length of survival, especially in the anaplastic category. We have analyzed the 1p/19q status of pediatric OGs and compared it with similar alterations in adult OGs. Paraffin sections from 26 pediatric OGs (21 WHO Grade II OGs; 2 anaplastic oligodendrogliomas [AOGs]; and 3 mixed oligo-astrocytomas [MOA]) were retrieved. Fluorescence in situ hybridization (FISH) was performed using probes spanning the 1p32 and 19q13 regions. In tumors from children 0 to 9 years of age (n = 15), none had any deletions on 1p or 19q, but 2 had polysomies for 1p and/or 19q. All are alive and 4 have had recurrences. In tumors from children >9 years, losses were identified on chromosomes 1p (5/11: 45%) and/or 19q (3/11; 27%), but to a much lesser extent than that observed in adult OGs. Tumors from 6 older patients also had polysomies for 1p and/or 19q. Although the majority of the older children are alive, 4 had recurrences. Curiously, 2 of the older children with AOGs had combined losses and polysomies on 1p and 19q, but responded poorly to treatment and died within a year. We conclude that alterations on 1p or 19q are infrequent in pediatric compared to adult OGs and are virtually absent in OGs presenting in the first decade of life. Compared to adults therefore, different genetic pathways are likely involved in the pathogenesis of most pediatric OGs. Genomic screening on a larger series is clearly indicated to delineate the unique molecular characteristics of these rare pediatric tumors.
AB - Oligodendrogliomas (OGs) are rare in children and have not been well characterized from a molecular viewpoint. In adults, losses on chromosomes 1p and/or 19q are common in "oligodendroglial" neoplasms and are highly associated with chemosensitivity and greater length of survival, especially in the anaplastic category. We have analyzed the 1p/19q status of pediatric OGs and compared it with similar alterations in adult OGs. Paraffin sections from 26 pediatric OGs (21 WHO Grade II OGs; 2 anaplastic oligodendrogliomas [AOGs]; and 3 mixed oligo-astrocytomas [MOA]) were retrieved. Fluorescence in situ hybridization (FISH) was performed using probes spanning the 1p32 and 19q13 regions. In tumors from children 0 to 9 years of age (n = 15), none had any deletions on 1p or 19q, but 2 had polysomies for 1p and/or 19q. All are alive and 4 have had recurrences. In tumors from children >9 years, losses were identified on chromosomes 1p (5/11: 45%) and/or 19q (3/11; 27%), but to a much lesser extent than that observed in adult OGs. Tumors from 6 older patients also had polysomies for 1p and/or 19q. Although the majority of the older children are alive, 4 had recurrences. Curiously, 2 of the older children with AOGs had combined losses and polysomies on 1p and 19q, but responded poorly to treatment and died within a year. We conclude that alterations on 1p or 19q are infrequent in pediatric compared to adult OGs and are virtually absent in OGs presenting in the first decade of life. Compared to adults therefore, different genetic pathways are likely involved in the pathogenesis of most pediatric OGs. Genomic screening on a larger series is clearly indicated to delineate the unique molecular characteristics of these rare pediatric tumors.
KW - Chromosome 19q
KW - Chromosome 1p
KW - Fluorescence in situ hybridization (FISH)
KW - Molecular genetics
KW - Pediatric oligodendroglioma
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U2 - 10.1093/jnen/62.5.530
DO - 10.1093/jnen/62.5.530
M3 - Article
C2 - 12769192
AN - SCOPUS:0037884872
SN - 0022-3069
VL - 62
SP - 530
EP - 537
JO - Journal of neuropathology and experimental neurology
JF - Journal of neuropathology and experimental neurology
IS - 5
ER -