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Objective:To investigate the clinic opathological features, treatment and prognosis of children newly diagnosed with ependymoma.Methods:Clinical data of 127 pediatric ependymoma (EPN) patients (0-16 years old) treated with tumor resection and postoperative radiotherapy at Xinhua Hospital Affiliated to Shanghai Jiao Tong University between 2001 and 2021 were retrospectively analyzed. Among them, 53 children were female and 74 were male. Local control (LR), event-free survival (EFS) and overall survival (OS) rates were analyzed by Kaplan-Meier method. The relationship between clinic opathological factors and clinical prognosis, and the effect of treatment on clinical prognosis of patients were analyzed by Cox proportional hazards model.Results:At a median follow-up time of 29 months (3-251 months), the 3-year OS and EFS rates were 89.5% and 71.5%, respectively. For patients undergoing incomplete resection followed by postoperative adjuvant radiotherapy, the 3-year LR, OS and EFS rates were 78.3%, 65.8% and 85.7%, respectively. A total of 43 children were aged <3 years old when diagnosed and 84 aged ≥3 years old. The interval time between surgery and radiotherapy in children aged <3 years old was 91 d, and 35.5 d in those aged ≥3 years old ( P<0.001). For patients <3 years old, the median EFS was 90 months when initiating radiotherapy within ≤70 d after surgery, compared to 43 months for those who initiated radiotherapy at >70 d after surgery ( P=0.053). According to fifth edition of the WHO classification of tumors of the central nervous system (WHO CNS5), 39 children were classified as posterior fossa ependymoma group A (PFA group). The OS and EFS rates in the PFA group were significantly less than those in other groups (3-year OS rate were 69.2% vs. 94.6%, P<0.001; 3-year EFS rate were 46.9% vs. 79.1%, P<0.001). In the PFA group, 12 patients received postoperative adjuvant chemotherapy, 14 did not receive chemotherapy, and whether chemotherapy was given was unknown in 13 cases. No significant differences were observed in OS and EFS between patients treated with and without chemotherapy ( P=0.260, P=0.730). Univariate Cox analysis showed that tumor location and WHO CNS5 molecular classification were significantly associated with EFS, and WHO CNS5 molecular classification was significantly correlated with OS. Multivariate Cox analysis showed that tumor location in the posterior fossa was an independent risk factor for EFS ( HR=2.72, 95% CI=1.1~6.71, P=0.03). Conclusions:Patients newly diagnosed with pediatric ependymoma can obtain favorable survival after surgery combined with postoperative adjuvant radiotherapy. Patients with residual tumors can achieve favorable LC and survival after postoperative adjuvant radiotherapy. Delaying of radiotherapy tends to lead to poor survival for patients aged <3 years old when diagnosed. Children in the PFA group obtain worse prognosis compared to their counterparts in other groups. The tumor location in the posterior fossa is an independent risk factor for pediatric ependymoma.
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Objective:To investigate the clinical features of pediatric patients with intracranial primary non-germinomatous germ cell tumors (NGGCT) and evaluate the treatment outcomes and prognostic factors of NGGCT.Methods:Clinical data of 40 children with NGGCT who were treated with radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received craniospinal irradiation (CSI). All children received platinum-based chemotherapy. Survival analysis was conducted using the Kaplan-Meier estimate. The prognostic factors were analyzed by log-rank test.Results:The primary sites were pineal gland, sellar / suprasellar region and basal ganglia. The median age of onset was 108 months (20-204 months). The median follow-up time was 33 months (8-131 months), and the 3-year and 5-year overall survival (OS) rates were 82.0%. The 3-year and 5-year progression-free survival (PFS) rates were 78.6% and 73.0%. Univariate analysis showed that increased alpha-fetoprotein (AFP) ( P=0.02), age at first diagnosis>10 years ( P=0.006), metastasis at first diagnosis ( P<0.001), and the pathological type (choriocarcinoma, yolk sac tumor and / or embryonal carcinoma) ( P=0.036) were independent adverse prognostic factors. Conclusions:Increased AFP, age>10 years at first diagnosis, tumor metastasis and pathological type were independent adverse prognostic factors of NGGCT. The overall prognosis of NGGCT children is worse than that of their counterparts with germinoma, and multidisciplinary intensive therapy is needed to improve survival.
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ObjectiveTo discuss dosimetric characteristics of an intensity-modulated radiotherapy (IMRT) technique for treating the chest wall and regional nodes as an integrated volume after modified radical mastectomy ( MRM ),and observe acute side-effects following irradiation.Methods From June 2009 to August 2010,75 patients were randomly enrolled.Of these,41 had left-sided breast cancer.Each eligible patient had a planning CT in treatment position,on which the chest wall,supraclavicular,and infraclavicular nodes,+/-internal mammary region,were contoured as an integrated volume.A muhi-beam IMRT plan was designed with the target either as a whole or two segments divided at below the clavicle head.A dose of 50 Gy in 25 fractions was prescribed to cover at least 90％ of the PTV.Internal mammary region was included in 31 cases.Dose volume histograms were used to evaluate the IMRT plans.The acute side effects were followed up regularly during and after irradiation.The independent two-sample t-test was used to compare the dosimetric parameters between integrated and segmented plans.ResultsPlanning design was completed for all patients,including 55 integrated and 20 segmented plans,with median number of beams of 8.The conformity index and homogeneity index was 1.43 ± 0.15 and 0.14 ± 0.02,respectively.Patients with internal mammary region included in PTV had higher homogeneity index PT.The percent volume of PTV receiving ＞ 110％ prescription dose was ＜ 5％.None of the dose constraints to normal structures was violated.There were statistically significant differences in the means of dosimetric parameters of PTV,such as Dmax,DmeanV107％,and V110％,between integrated and segmented plans (t=2.19 -2.53,P=0.013-0.031 ).≥ grade 2 radiation dermatitis was identified in 3 2 patients ( grade 2 in 2 2 patients,grade 3 in 10 patients ),mostly occurred within 1 - 2 weeks after treatment.The sites of moist desquamation were anterior axillary fold (27/37) and chest wall (10/37).Only 2 patients developed grade 2 radiation pneumonitis.Conclusions The IMRT technique applied after MRM with integrated locoregional target volume is dosimetrically feasible,and the treatment was proved to be well-tolerated by most patients.
