TY - JOUR
T1 - Association between DNA content and tumor suppressor gene expression and aggressiveness of atypical teratoid/rhabdoid tumors
AU - Berrak, Su Gulsun
AU - Ozek, Memet M.
AU - Canpolat, Cengiz
AU - Daǧçinar, Adnan
AU - Sav, Aydin
AU - El-Naggar, Adel
AU - Langford, Lauren A.
N1 - Funding Information:
Acknowledgement This work was supported by National Institutes of Health Grants RO1 CA56041 and P01 CA55261 (L.A.L.).
PY - 2002/10
Y1 - 2002/10
N2 - Objects: Atypical teratoid/rhabdoid tumors “AT/RT” are aggressive neoplasms that afflict infants and young children. The objective of this retrospective study was to determine the association between DNA content “DNA ploidy, cell cycle analysis”, tumor suppressor gene “p53, pRb, p16, and MMAC/PTEN” expression and the biologic aggressiveness of these tumors. Methods: Eight tumors from 7 patients “1 girl, 6 boys; median age 4±6.7 months” were studied. Two patients had DNA aneuploidy and 5 patients manifested diploid DNA content at diagnosis. The proliferative index of the tumors ranged from 10% to 28% “median, 12±6.4%”. The single tumor with a low proliferative index “i.e., <10%” was aneuploid. Immunohistochemical evaluation of p53, pRb, p16, and MMAC/PTEN expression patterns showed that most of the tumors contained more cells with abnormal pRb and p16 expression than cells with abnormal p53 and MMAC/PTEN expression. Expression of tumor suppressor genes, however, was inhomogeneous. Conclusion: Our findings led us to conclude that AT/RT of childhood is characterized by a high proliferative index and DNA aneuploidy. The high expression of abnormal pRb and p16 and the low expression of abnormal p53 and MMAC/PTEN indicate alteration of the G1-to-S phase step in the cell cycle, which could be an explanation for the aggressive nature of these tumors.
AB - Objects: Atypical teratoid/rhabdoid tumors “AT/RT” are aggressive neoplasms that afflict infants and young children. The objective of this retrospective study was to determine the association between DNA content “DNA ploidy, cell cycle analysis”, tumor suppressor gene “p53, pRb, p16, and MMAC/PTEN” expression and the biologic aggressiveness of these tumors. Methods: Eight tumors from 7 patients “1 girl, 6 boys; median age 4±6.7 months” were studied. Two patients had DNA aneuploidy and 5 patients manifested diploid DNA content at diagnosis. The proliferative index of the tumors ranged from 10% to 28% “median, 12±6.4%”. The single tumor with a low proliferative index “i.e., <10%” was aneuploid. Immunohistochemical evaluation of p53, pRb, p16, and MMAC/PTEN expression patterns showed that most of the tumors contained more cells with abnormal pRb and p16 expression than cells with abnormal p53 and MMAC/PTEN expression. Expression of tumor suppressor genes, however, was inhomogeneous. Conclusion: Our findings led us to conclude that AT/RT of childhood is characterized by a high proliferative index and DNA aneuploidy. The high expression of abnormal pRb and p16 and the low expression of abnormal p53 and MMAC/PTEN indicate alteration of the G1-to-S phase step in the cell cycle, which could be an explanation for the aggressive nature of these tumors.
KW - Atypical teratoid/rhabdoid tumor
KW - MMAC/PTEN
KW - Tumor suppressor genes
KW - p16
KW - p53
KW - pRb
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U2 - 10.1007/s00381-002-0633-9
DO - 10.1007/s00381-002-0633-9
M3 - Article
C2 - 12382173
AN - SCOPUS:0036818076
SN - 0256-7040
VL - 18
SP - 485
EP - 491
JO - Child's Nervous System
JF - Child's Nervous System
IS - 9-10
ER -