Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006.
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Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006. / Gerber, Nicolas U; von Hoff, Katja; von Bueren, André; Treulieb, Wiebke; Treulieb, Wiebke; Warmuth-Metz, Monika; Pietsch, Torsten; Soerensen, Niels; Faldum, Andreas; Emser, Angela; Schlegel, Paul G; Deinlein, Frank; Rutkowski, Stefan; Rutkowski, Stefan.
In: J NEURO-ONCOL, Vol. 102, No. 3, 3, 2011, p. 459-469.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006.
AU - Gerber, Nicolas U
AU - von Hoff, Katja
AU - von Bueren, André
AU - Treulieb, Wiebke
AU - Treulieb, Wiebke
AU - Warmuth-Metz, Monika
AU - Pietsch, Torsten
AU - Soerensen, Niels
AU - Faldum, Andreas
AU - Emser, Angela
AU - Schlegel, Paul G
AU - Deinlein, Frank
AU - Rutkowski, Stefan
AU - Rutkowski, Stefan
PY - 2011
Y1 - 2011
N2 - Ependymoblastoma is a rare malignant brain tumor of early childhood. Data on clinical behavior and optimal treatment strategies are scarce. We report on 11 consecutively treated children with centrally confirmed diagnosis of CNS ependymoblastoma, registered between February 1994 and October 2006 to the prospective GPOH-HIT multicenter brain tumor trials, and treated by multimodal regimens. Median age at diagnosis was 3.5 years (range, 1.8-5.6 years), and the median follow-up of survivors was 5.9 years (range, 2.2-12.7 years). Initial stage was M0 in 9, and M0/1 (no cerebrospinal fluid examination done) in 2 patients. Gross-total tumor resection was achieved in 7 patients, incomplete resection in 4 patients. Further primary therapy included chemotherapy in all patients, craniospinal radiotherapy in 5 patients and high-dose chemotherapy in 2 patients. Tumor response to chemotherapy was observed in 1 of 4 evaluable patients. Tumor progression occurred in 7 patients after a median time of 5.0 months (range, 2.5-19.2 months). Five-year progression-free survival was 36.4% (±14.5%), 5-year overall survival 30.3% (±15.9%). Of 4 survivors, 3 had gross-total tumor resection, and all were treated by either craniospinal radiotherapy and/or high-dose chemotherapy with autologous blood stem cell rescue. Prognosis of children with ependymoblastoma is poor, but sustained remissions have been achieved after multimodal treatment. Considerable diagnostic discrepancies between local and central pathologists underscore the importance of central review. Further studies are needed to improve survival of children with this rare malignant central nervous system tumor.
AB - Ependymoblastoma is a rare malignant brain tumor of early childhood. Data on clinical behavior and optimal treatment strategies are scarce. We report on 11 consecutively treated children with centrally confirmed diagnosis of CNS ependymoblastoma, registered between February 1994 and October 2006 to the prospective GPOH-HIT multicenter brain tumor trials, and treated by multimodal regimens. Median age at diagnosis was 3.5 years (range, 1.8-5.6 years), and the median follow-up of survivors was 5.9 years (range, 2.2-12.7 years). Initial stage was M0 in 9, and M0/1 (no cerebrospinal fluid examination done) in 2 patients. Gross-total tumor resection was achieved in 7 patients, incomplete resection in 4 patients. Further primary therapy included chemotherapy in all patients, craniospinal radiotherapy in 5 patients and high-dose chemotherapy in 2 patients. Tumor response to chemotherapy was observed in 1 of 4 evaluable patients. Tumor progression occurred in 7 patients after a median time of 5.0 months (range, 2.5-19.2 months). Five-year progression-free survival was 36.4% (±14.5%), 5-year overall survival 30.3% (±15.9%). Of 4 survivors, 3 had gross-total tumor resection, and all were treated by either craniospinal radiotherapy and/or high-dose chemotherapy with autologous blood stem cell rescue. Prognosis of children with ependymoblastoma is poor, but sustained remissions have been achieved after multimodal treatment. Considerable diagnostic discrepancies between local and central pathologists underscore the importance of central review. Further studies are needed to improve survival of children with this rare malignant central nervous system tumor.
M3 - SCORING: Zeitschriftenaufsatz
VL - 102
SP - 459
EP - 469
JO - J NEURO-ONCOL
JF - J NEURO-ONCOL
SN - 0167-594X
IS - 3
M1 - 3
ER -