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
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Italy.
Pediatric Unit, University-Hospital of Padua, Padova, Italy.
Pediatr Blood Cancer. 2017 Nov;64(11). doi: 10.1002/pbc.26568. Epub 2017 Apr 27.
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.
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.
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%).
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.
恶性卵巢生殖细胞肿瘤（MOGCT）预后良好，治疗旨在以尽可能低的治疗相关发病率取得疗效。本研究的目的是评估儿童MOGCT患者的治疗结果。
根据患者分期进行治疗：I期患者接受手术及监测；II期（三个周期）、III期和IV期（三个周期）患者采用改良博来霉素-依托泊苷-顺铂（BEP）方案，并对残留病灶进行手术。
共纳入77例患者（中位年龄11.8岁），其中26例为无性细胞瘤（Dysg），13例为未成熟畸胎瘤且血清甲胎蛋白水平升高（IT + AFP），38例为非无性细胞瘤（Non-Dysg），分期如下：27例I期，13例II期，32例III期，5例IV期。在I期可评估患者中（5年无事件生存率[EFS]为72.1%[95%CI：56.4 - 92.1%]；5年总生存率[OS]为100%），7例复发（3例Dysg患者和4例Non-Dysg患者），通过化疗（3例患者加手术）挽救。在II - IV期可评估患者中，48例（98%）在化疗±手术后达到完全缓解，1例（IT + AFP，IV期）病情进展。在整个系列中（中位随访80个月），5年OS和EFS分别为98.5%（95%CI：95.6 - 100%）和84.5%（95%CI：76.5 - 93.5%）。
我们证实了MOGCT的良好治疗结果。关于手术分期、I期Non-Dysg的监测、IT + AFP的管理以及最合适的BEP方案，缺乏有力的数据。作为儿科肿瘤学家，我们支持在适当的手术分期后进行监测，前提是病例由专业儿科中心的专家管理。
