Malignant ovarian germ cell tumors in pediatric patients: TheAIEOP (Associazione Italiana Ematologia Oncologia Pediatrica)study

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.