TY - JOUR
T1 - Malignant germ cell tumors in childhood
T2 - Results of the first Italian cooperative study "TCG 91"
AU - Curto, Margherita Lo
AU - Lumia, Francesca
AU - Alaggio, Rita
AU - Cecchetto, Giovanni
AU - Almasio, Piero
AU - Indolfi, Paolo
AU - Siracusa, Fortunato
AU - Bagnulo, Serenella
AU - De Bernardi, Bruno
AU - De Laurentis, Tina
AU - Di Cataldo, Andrea
AU - Tamaro, Paolo
PY - 2003/11/1
Y1 - 2003/11/1
N2 - Background and Aims. About 20% of patients with germ cell tumor (GCT) are still resistant to therapy. To investigate which features are present in resistant patients, a multicenter study on GCT in children was undertaken to correlate clinical and laboratory parameters with the outcome. Methods. Patients aged less than 16 years, with histologically proven extracranial GCT were included. Results. Ninety-five patients (median age 33 months, 45 males) were eligible. The site of the primary tumor was gonadal in 59, extragonadal in 36. The stage was I in 39; II in 5; IIIa (microscopic residue) in 7; IIIb (macroscopic residue) in 16; IIIc (unresectable) in 13; IV in 15. The treatment was surgery alone in 31; surgery plus radiotherapy in 1; chemotherapy ± surgery in 63. Post-chemotherapy resection in 19 (10 complete, 9 partial). The chemotherapy regimen was carboplatin 400 mg/m2/day on days 1, 2; etoposide 150 mg/m2/day on days 1, 2; ifosfamide 1,500 mg/m 2/day on days 21, 22; dactinomycin 1.5 mg/m2/day on day 21; vincristine 1.5 mg/m2/day on day 21. Three patients died because of toxicity and two non-responders (to primary chemotherapy), died of progression; among the remaining 90 patients 20 relapsed, 9 are in second remission, 2 are alive with disease, and 9 died of disease progression (one from progression and intracranial hemorrhage). Overall survival was 82.7% and event-free survival: 71.5%. Survival according to: (a) site: testis: 100%; ovary: 88%; sacrococcyx: 69.6%; other sites: 33.3% (P <0.001); (b) stage: I and II: 100%; IIIa: 83.3%; IIIb: 84.6%; IIIc: 60.6%; IV: 53.2% (P <0.001); (c) AFP levels: normal: 85.5%; 429,470 ng/ml: 84.6%; ≥10,000 ng/ml: 58.7% (P = 0.02). All the pts who had complete resection of the primary tumor at diagnosis or at delayed surgery, remained in remission. Conclusions. Multivariate analysis showed that the primary site of tumor was the only independent prognostic factor for survival and EFS.
AB - Background and Aims. About 20% of patients with germ cell tumor (GCT) are still resistant to therapy. To investigate which features are present in resistant patients, a multicenter study on GCT in children was undertaken to correlate clinical and laboratory parameters with the outcome. Methods. Patients aged less than 16 years, with histologically proven extracranial GCT were included. Results. Ninety-five patients (median age 33 months, 45 males) were eligible. The site of the primary tumor was gonadal in 59, extragonadal in 36. The stage was I in 39; II in 5; IIIa (microscopic residue) in 7; IIIb (macroscopic residue) in 16; IIIc (unresectable) in 13; IV in 15. The treatment was surgery alone in 31; surgery plus radiotherapy in 1; chemotherapy ± surgery in 63. Post-chemotherapy resection in 19 (10 complete, 9 partial). The chemotherapy regimen was carboplatin 400 mg/m2/day on days 1, 2; etoposide 150 mg/m2/day on days 1, 2; ifosfamide 1,500 mg/m 2/day on days 21, 22; dactinomycin 1.5 mg/m2/day on day 21; vincristine 1.5 mg/m2/day on day 21. Three patients died because of toxicity and two non-responders (to primary chemotherapy), died of progression; among the remaining 90 patients 20 relapsed, 9 are in second remission, 2 are alive with disease, and 9 died of disease progression (one from progression and intracranial hemorrhage). Overall survival was 82.7% and event-free survival: 71.5%. Survival according to: (a) site: testis: 100%; ovary: 88%; sacrococcyx: 69.6%; other sites: 33.3% (P <0.001); (b) stage: I and II: 100%; IIIa: 83.3%; IIIb: 84.6%; IIIc: 60.6%; IV: 53.2% (P <0.001); (c) AFP levels: normal: 85.5%; 429,470 ng/ml: 84.6%; ≥10,000 ng/ml: 58.7% (P = 0.02). All the pts who had complete resection of the primary tumor at diagnosis or at delayed surgery, remained in remission. Conclusions. Multivariate analysis showed that the primary site of tumor was the only independent prognostic factor for survival and EFS.
KW - Carboplatin
KW - Malignant germ cell tumors
KW - Risk factors in malignant germ cell tumors
KW - Seminoma
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U2 - 10.1002/mpo.10324
DO - 10.1002/mpo.10324
M3 - Article
C2 - 14515380
AN - SCOPUS:0141960354
VL - 41
SP - 417
EP - 425
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 5
ER -