RESUMO
We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan-Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/-CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.
RESUMO
Due to the rarity of high-grade gliomas (HGG) in children, data on this topic are scarce. The study aimed to investigate the long-term results of treatment of children with HGG and to identify factors related to better survival. We performed a retrospective analysis of patients treated for HGG who had the main tumor located outside the brainstem. The evaluation of factors that correlated with better survival was performed with the Cox proportional-hazard model. Survival was estimated with the Kaplan-Meier method. The study group consisted of 82 consecutive patients. All of them underwent surgery as primary treatment. Chemotherapy was applied in 93% of children with one third treated with temozolomide. After or during the systemic treatment, 79% of them received radiotherapy with a median dose of 54 Gy. Median follow-up was 122 months, and during that time, 59 patients died. One-, 2-, 5-, and 10-year overall survival was 78%, 48%, 30% and 17%, respectively. Patients with radical (R0) resection and temozolomide-based chemotherapy had better overall survival. Progression-free survival was better in patients after R0 resection and radical radiotherapy. The best outcome in HGG patients was observed in patients after R0 resection with immediate postoperative temozolomide-based chemotherapy and radical radiotherapy.
RESUMO
OBJECTIVES: The aim of this population-based, retrospective study was to analyze biological and clinical features and treatment results in children diagnosed with MPAL in all Polish pediatric oncology centers between 2007 and 2018. METHODS: Among 2893 children and adolescents diagnosed and treated for acute leukemia, 39 (1.35%) patients fulfilled the WHO criteria of MPAL. The T/myeloid phenotype was most prevalent. RESULTS: Cytogenetics findings were seen in 2 (5.1%), while chromosomal abnormalities were found in 14 (35.9%) patients. Thirty-two patients achieved CR-1, including 23 (92.0%) treated with ALL-directed chemotherapy and 9 (64.3%) treated with AML-type induction regimens. Within these patients, 4 (12.5%) died due to treatment-related complications and 11 (34.4%) relapsed. Nineteen (63.3%) patients underwent allo-HSCT in CR-1 and 14 (73.7%) of them have been in CR-1. In total, 17 (43.6%) patients remain in CR-1 for 1-12 years, including 14 (58.3%) with T/myeloid MPAL. The 5-year pOS and pEFS were 51.8% and 44.2%, respectively. The overall survival for ALL-directed therapy was significantly better than the one for AML-type chemotherapy (P = .001). It was also better for patients who underwent HSCT in CR-1 (P = .001). CONCLUSIONS: The prognosis of MPAL is unsatisfactory, but initial treatment with ALL-directed chemotherapy consolidated with allo-HSCT improves the outcomes in MPAL.