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Malignant ovarian germ cell tumors in pediatric patients: The AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) study.
Terenziani, M; Bisogno, G; Boldrini, R; Cecchetto, G; Conte, M; Boschetti, L; De Pasquale, M D; Biasoni, D; Inserra, A; Siracusa, F; Basso, M E; De Leonardis, F; Di Pinto, D; Barretta, F; Spreafico, F; D'Angelo, P.
Afiliação
  • Terenziani M; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
  • Bisogno G; Pediatric Unit, University-Hospital of Padua, Padova, Italy.
  • Boldrini R; Pathology Unit, Ospedale Pediatrico Bambino Gesù-IRCCS. Roma, Italy.
  • Cecchetto G; Pediatric Surgery Unit, University-Hospital of Padua, Padova, Italy.
  • Conte M; Oncology Unit, Ospedale Pediatrico G. Gaslini, Genova, Italy.
  • Boschetti L; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
  • De Pasquale MD; Hematology/Oncology Department, Ospedale Pediatrico Bambino Gesù-IRCCS, Roma, Italy.
  • Biasoni D; Pediatric Surgery Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
  • Inserra A; Pediatric Surgery Department, Ospedale Pediatrico Bambino Gesù-IRCCS, Roma, Italy.
  • Siracusa F; Pediatric Surgery Department, Università of Palermo, Palermo, Italy.
  • Basso ME; Hematology/Oncology Unit, Ospedale Infantile Regina Margherita, Torino, Italy.
  • De Leonardis F; Division of Pediatric Hematology-Oncology, University of Bari, Italy.
  • Di Pinto D; Pediatric Oncology Unit, Seconda Università, Napoli, Italy.
  • Barretta F; Unit of Clinical Epidemiology and Trial Organization, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
  • Spreafico F; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
  • D'Angelo P; Hematology/Oncology Unit, A.R.N.A.S Civico Di Cristina e Benfratelli, Palermo, Italy.
Pediatr Blood Cancer ; 64(11)2017 Nov.
Article em En | MEDLINE | ID: mdl-28449306
ABSTRACT

OBJECTIVE:

Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and the treatment aims to achieve results with the least possible treatment-related morbidity. The aim of this 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 38 with 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 patients with Non-Dysg) and were rescued with chemotherapy (plus surgery in three patients). Among the evaluable 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-up 80 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 surgical staging, surveillance for Non-Dysg with stage I, the management of IT + AFP, and the most appropriate BEP regimen. As pediatric oncologists, we support the role of surveillance after proper surgical staging providing cases are managed by experts at specialized pediatric centers.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Neoplasias Ovarianas / Protocolos de Quimioterapia Combinada Antineoplásica / Neoplasias Embrionárias de Células Germinativas Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Ano de publicação: 2017 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Neoplasias Ovarianas / Protocolos de Quimioterapia Combinada Antineoplásica / Neoplasias Embrionárias de Células Germinativas Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Ano de publicação: 2017 Tipo de documento: Article