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

Fortunato Siracusa, Paolo D'Angelo, Di Pinto, Alessandro Inserra, Biasoni, De Leonardis, Boschetti, Barretta, Monica Terenziani, Maria Debora De Pasquale, Gianni Bisogno, Filippo Spreafico, Giovanni Cecchetto, Renata Boldrini, Massimo Conte, Basso

    Risultato della ricerca: Articlepeer review

    10 Citazioni (Scopus)

    Abstract

    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.
    Lingua originaleEnglish
    pagine (da-a)1-8
    Numero di pagine8
    RivistaPEDIATRIC BLOOD & CANCER
    Volume9
    Stato di pubblicazionePublished - 2017

    All Science Journal Classification (ASJC) codes

    • Pediatrics, Perinatology, and Child Health
    • Hematology
    • Oncology

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