Background: Neuroblastoma (NB) is remarkable for its wide spectrum of clinical behavior and biological characteristics in relation to outcome. The use of aggressive therapy, including autologous hematopoietic stem cel...Background: Neuroblastoma (NB) is remarkable for its wide spectrum of clinical behavior and biological characteristics in relation to outcome. The use of aggressive therapy, including autologous hematopoietic stem cell transplantation (HSCT) and the addition of isoretionin (cis-Retinoic Acid/cis-RA), has increased survival rates of patients with advanced disease. Methods: Pediatric 271 newly diagnosed high risk NB patients were prospectively enrolled into the study. Patients received neoadjuvant chemotherapy of alternating cycles: [cyclophosphamide, doxorubicin, vincristine (CAdO)] and [etoposide, carboplatin]. Intensification courses of “ICE” (ifosfamide, carboplatin, and etoposide) regimen were administered to patients with bone marrow (BM) residual infiltration. Whenever safely feasible, complete surgical resection or debulking of the primary tumor was attempted for patients achieving partial response. Eligible patients underwent HSCT, while radiation therapy to the primary and metastatic sites, as well as maintenance with cis-RA was given for 6 months. Results: The median age of our patients was 2.8 years with male to female ratio of 1.65:1. At 4 years, the overall and event free survivals were 33.7% and 23.3% for the entire group under study, with significantly higher rates (42.7% and 35.6%, respectively) for HSCT patients (n = 94;p 0.001). The outcome was also significantly correlated with response to induction therapy, pathological subtype, as well as other variables. Conclusion: Myeloablative therapy followed by stem cell rescue is regarded as the most important goal of high risk NB treatment to improve survival till present. Each of consolidation HSCT, post induction disease status, as well as international neuroblastoma pathology classification (INPC) subtype was an independent predictive variable of survival. A collaborative effort with an emphasis on biologic characteristics of aggressive disease and tailored therapy needs to be strengthened to further our understanding of this disease.展开更多
Objective：This study was to review the efficacy of surgical resections in different clinical situations for a better understanding of the meaning of surgery in the treatment of neuroblastoma （NB）.Data Sources：The ...Objective：This study was to review the efficacy of surgical resections in different clinical situations for a better understanding of the meaning of surgery in the treatment of neuroblastoma （NB）.Data Sources：The online database ScienceDirect （2016-2018） was utilized.The search was conducted using the keywords ＂neuroblastoma,＂ ＂neuroblastoma resection,＂ ＂neuroblastoma surgery,＂ and ＂high-risk neuroblastoma.＂Study Selection：We retrospectively analyzed of patients who underwent surgical resections in different clinical situations.The article included findings from selected relevant randomized controlled trials,systematic reviews,and meta-analyses or good-quality observational studies.Abstracts only,letters,and editorial notes were excluded.Full-text articles and abstracts were extracted and reviewed to identify key articles discussing surgery management of NB,which were then selected for critical analysis.Results：A total of 7800 English language articles were found containing references to NB （2016-2018）.The 163 articles were searched which were related to the surgical treatment of NB （2016-2018）.Through the analysis of these important articles,we found that the treatments of NB at low-and intermediate-risk groups were basically the same.High-risk patients remained controversial.Conclusions：NB prognosis varies tremendously based on the stage and biologic features of the tumor.After reviewing the relevant literature,patients with low-risk disease are often managed with surgical resection or observation alone with tumors likely to spontaneously regress that are not causing symptoms.Intermediate patients are treated with chemotherapy with the number of cycles depending on their response as well as surgical resection of the primary tumor.High-risk patients remain controversial.Multidisciplinary intensive treatment is essential,especially for patients who received subtotal tumor resection.Minimally invasive surgery for the treatment of NBs without image-defined risk factors in low-to high-risk patients is safe and feasible and does not compromise the treatment outcome.We conclude that 〉90% resection of the primary tumor is both feasible and safe in most patients with high-risk NB.New targeted therapies are crucial to improve survival.展开更多
文摘Background: Neuroblastoma (NB) is remarkable for its wide spectrum of clinical behavior and biological characteristics in relation to outcome. The use of aggressive therapy, including autologous hematopoietic stem cell transplantation (HSCT) and the addition of isoretionin (cis-Retinoic Acid/cis-RA), has increased survival rates of patients with advanced disease. Methods: Pediatric 271 newly diagnosed high risk NB patients were prospectively enrolled into the study. Patients received neoadjuvant chemotherapy of alternating cycles: [cyclophosphamide, doxorubicin, vincristine (CAdO)] and [etoposide, carboplatin]. Intensification courses of “ICE” (ifosfamide, carboplatin, and etoposide) regimen were administered to patients with bone marrow (BM) residual infiltration. Whenever safely feasible, complete surgical resection or debulking of the primary tumor was attempted for patients achieving partial response. Eligible patients underwent HSCT, while radiation therapy to the primary and metastatic sites, as well as maintenance with cis-RA was given for 6 months. Results: The median age of our patients was 2.8 years with male to female ratio of 1.65:1. At 4 years, the overall and event free survivals were 33.7% and 23.3% for the entire group under study, with significantly higher rates (42.7% and 35.6%, respectively) for HSCT patients (n = 94;p 0.001). The outcome was also significantly correlated with response to induction therapy, pathological subtype, as well as other variables. Conclusion: Myeloablative therapy followed by stem cell rescue is regarded as the most important goal of high risk NB treatment to improve survival till present. Each of consolidation HSCT, post induction disease status, as well as international neuroblastoma pathology classification (INPC) subtype was an independent predictive variable of survival. A collaborative effort with an emphasis on biologic characteristics of aggressive disease and tailored therapy needs to be strengthened to further our understanding of this disease.
文摘Objective：This study was to review the efficacy of surgical resections in different clinical situations for a better understanding of the meaning of surgery in the treatment of neuroblastoma （NB）.Data Sources：The online database ScienceDirect （2016-2018） was utilized.The search was conducted using the keywords ＂neuroblastoma,＂ ＂neuroblastoma resection,＂ ＂neuroblastoma surgery,＂ and ＂high-risk neuroblastoma.＂Study Selection：We retrospectively analyzed of patients who underwent surgical resections in different clinical situations.The article included findings from selected relevant randomized controlled trials,systematic reviews,and meta-analyses or good-quality observational studies.Abstracts only,letters,and editorial notes were excluded.Full-text articles and abstracts were extracted and reviewed to identify key articles discussing surgery management of NB,which were then selected for critical analysis.Results：A total of 7800 English language articles were found containing references to NB （2016-2018）.The 163 articles were searched which were related to the surgical treatment of NB （2016-2018）.Through the analysis of these important articles,we found that the treatments of NB at low-and intermediate-risk groups were basically the same.High-risk patients remained controversial.Conclusions：NB prognosis varies tremendously based on the stage and biologic features of the tumor.After reviewing the relevant literature,patients with low-risk disease are often managed with surgical resection or observation alone with tumors likely to spontaneously regress that are not causing symptoms.Intermediate patients are treated with chemotherapy with the number of cycles depending on their response as well as surgical resection of the primary tumor.High-risk patients remain controversial.Multidisciplinary intensive treatment is essential,especially for patients who received subtotal tumor resection.Minimally invasive surgery for the treatment of NBs without image-defined risk factors in low-to high-risk patients is safe and feasible and does not compromise the treatment outcome.We conclude that 〉90% resection of the primary tumor is both feasible and safe in most patients with high-risk NB.New targeted therapies are crucial to improve survival.
