TY - JOUR
T1 - Malignant ovarian germ cell tumors in pediatric patients: TheAIEOP (Associazione Italiana Ematologia Oncologia Pediatrica)study
AU - Siracusa, Fortunato
AU - D'Angelo, Paolo
AU - Di Pinto, null
AU - Inserra, Alessandro
AU - Biasoni, null
AU - De Leonardis, null
AU - De Leonardis, null
AU - Boschetti, null
AU - Barretta, null
AU - Terenziani, Monica
AU - De Pasquale, Maria Debora
AU - Bisogno, Gianni
AU - Spreafico, Filippo
AU - Cecchetto, Giovanni
AU - Boldrini, Renata
AU - Conte, Massimo
AU - Basso, null
PY - 2017
Y1 - 2017
N2 - Objective: Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and thetreatment aims to achieve results with the least possible treatment-related morbidity. The aim ofthis study was to assess the outcomes of pediatric patients with MOGCT.Methods: Patients were treated according to their stage: surgery and surveillance for stage I;a modified bleomycin–etoposide–cisplatin (BEP) regimen for stages II (three cycles), III, and IV(three cycles) with surgery on residual disease.Results: Seventy-seven patients were enrolled (median age 11.8 years), 26 with dysgerminoma(Dysg), 13 with immature teratoma and elevated serum alpha-fetoprotein levels (IT+AFP), and 38with nondysgeminoma (Non-Dysg) staged as follows: 27 stage I, 13 stage II, 32 stage III, 5 stage IV.Among evaluable patients in stage I (5-year event-free survival [EFS] 72.1% [95% CI: 56.4–92.1%];5-year overall survival [OS] 100%), seven relapsed (three patients with Dysg and four patientswith Non-Dysg) and were rescuedwith chemotherapy (plus surgery in three patients). Among theevaluable patients with stages II–IV, 48 (98%) achieved complete remission after chemotherapy± surgery, one (IT + AFP, stage IV) had progressive disease. In the whole series (median follow-up80 months), the 5-year OS and EFS were 98.5% (95% CI: 95.6–100%) and 84.5% (95% CI: 76.5–93.5%).Conclusions:We confirm the excellent outcome for MOGCT. Robust data are lacking on surgicalstaging, surveillance for Non-Dysg with stage I, the management of IT + AFP, and the most appropriateBEP regimen. As pediatric oncologists,we support the role of surveillance after proper surgicalstaging providing cases are managed by experts at specialized pediatric centers.
AB - Objective: Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and thetreatment aims to achieve results with the least possible treatment-related morbidity. The aim ofthis study was to assess the outcomes of pediatric patients with MOGCT.Methods: Patients were treated according to their stage: surgery and surveillance for stage I;a modified bleomycin–etoposide–cisplatin (BEP) regimen for stages II (three cycles), III, and IV(three cycles) with surgery on residual disease.Results: Seventy-seven patients were enrolled (median age 11.8 years), 26 with dysgerminoma(Dysg), 13 with immature teratoma and elevated serum alpha-fetoprotein levels (IT+AFP), and 38with nondysgeminoma (Non-Dysg) staged as follows: 27 stage I, 13 stage II, 32 stage III, 5 stage IV.Among evaluable patients in stage I (5-year event-free survival [EFS] 72.1% [95% CI: 56.4–92.1%];5-year overall survival [OS] 100%), seven relapsed (three patients with Dysg and four patientswith Non-Dysg) and were rescuedwith chemotherapy (plus surgery in three patients). Among theevaluable patients with stages II–IV, 48 (98%) achieved complete remission after chemotherapy± surgery, one (IT + AFP, stage IV) had progressive disease. In the whole series (median follow-up80 months), the 5-year OS and EFS were 98.5% (95% CI: 95.6–100%) and 84.5% (95% CI: 76.5–93.5%).Conclusions:We confirm the excellent outcome for MOGCT. Robust data are lacking on surgicalstaging, surveillance for Non-Dysg with stage I, the management of IT + AFP, and the most appropriateBEP regimen. As pediatric oncologists,we support the role of surveillance after proper surgicalstaging providing cases are managed by experts at specialized pediatric centers.
UR - http://hdl.handle.net/10447/226837
M3 - Article
VL - 9
SP - 1
EP - 8
JO - PEDIATRIC BLOOD & CANCER
JF - PEDIATRIC BLOOD & CANCER
SN - 1545-5009
ER -