Primary central nervous system germ cell tumors(CNS-GCTs) in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese chi...Primary central nervous system germ cell tumors(CNS-GCTs) in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years(range, 2.7 to 18.0 years) at diagnosis; 43(75.4%) had non-germinomatous germ cell tumors(NGGCTs) and 14(24.6%) had germinomas; 44(77.2%) had localized disease and 13(22.8%) had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB(cisplatin, etoposide, and bleomycin) protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months(range, 1.2 to 139 months). Fourteen patients died of relapse or disease progression. The 3-year event-free survival(EFS) and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs(P = 0.064). The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively(P = 0.042). Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.展开更多
Objective The aim of this study is to report the treatment result of childhood excranial malignant germ cell tumors and discuss the experience for recurrent and refractory cases treatment from our center.Methods We ha...Objective The aim of this study is to report the treatment result of childhood excranial malignant germ cell tumors and discuss the experience for recurrent and refractory cases treatment from our center.Methods We have retrospectively analyzed 58 extracranial malignant germ cell tumor patients treated with surgery and chemotherapy from our center over a 9-year period.Another 14 recurrent and refractory cases referred from other centers were added to the study for salvage approach.We evaluated the treatment results for primary cases and relapsed cases with a median follow-up of 61.5 months.Several factors were analysed to evaluate their power to the outcome of these cases.Results The 5-year event-free and overall survival for primary cases were 74.1±5.7%and 86.2±4.5%,respectively.25 recurrent or refractory cases entered the salvage approach study,and 17 patients were alive till the end of follow-up.We demonstrated superior survival outcome for those with successful local control through pre-operative and post-operative radiotherapy,second-look surgery and multi-drug secondline chemotherapies.Conclusion The outcome for childhood extracranial malignant germ cell tumors is generally favorable.For recurrent and refractory cases,multi-modality treatment approaches including radiotherapy,salvage chemotherapy and secondlook surgery are important for better local control.展开更多
Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fos...Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed.And a 4-year-old boy diagnosed with EST in our hospital was reported.The clinical manifestations,therapy,pathologic features,and prognosis of these cases were analyzed.Results Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review,including six cases searched from the PubMed and one case from our hospital.Six patients were boy and one patient’s gender was not available from the report.Ages ranged from 1 to 5 years(mean 3.14 years).The mean tumor size in our cohort was 4.4 cm.Six cases came from East Asia.Schiller-Duval bodies were found in all seven neoplasms.All tumors were positive for alpha-fetoprotein.The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy.The mean follow-up time was 24.4 months(range 5-52 months).Six tumors were totally removed,and four of them recurred.Three cases died including one whose tumor was partially removed.Conclusions The serum alpha-fetoprotein level is well correlated with the severity of the tumor.A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa.The prognosis of extragonadal intracranial EST is poor.展开更多
文摘Primary central nervous system germ cell tumors(CNS-GCTs) in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years(range, 2.7 to 18.0 years) at diagnosis; 43(75.4%) had non-germinomatous germ cell tumors(NGGCTs) and 14(24.6%) had germinomas; 44(77.2%) had localized disease and 13(22.8%) had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB(cisplatin, etoposide, and bleomycin) protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months(range, 1.2 to 139 months). Fourteen patients died of relapse or disease progression. The 3-year event-free survival(EFS) and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs(P = 0.064). The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively(P = 0.042). Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.
文摘Objective The aim of this study is to report the treatment result of childhood excranial malignant germ cell tumors and discuss the experience for recurrent and refractory cases treatment from our center.Methods We have retrospectively analyzed 58 extracranial malignant germ cell tumor patients treated with surgery and chemotherapy from our center over a 9-year period.Another 14 recurrent and refractory cases referred from other centers were added to the study for salvage approach.We evaluated the treatment results for primary cases and relapsed cases with a median follow-up of 61.5 months.Several factors were analysed to evaluate their power to the outcome of these cases.Results The 5-year event-free and overall survival for primary cases were 74.1±5.7%and 86.2±4.5%,respectively.25 recurrent or refractory cases entered the salvage approach study,and 17 patients were alive till the end of follow-up.We demonstrated superior survival outcome for those with successful local control through pre-operative and post-operative radiotherapy,second-look surgery and multi-drug secondline chemotherapies.Conclusion The outcome for childhood extracranial malignant germ cell tumors is generally favorable.For recurrent and refractory cases,multi-modality treatment approaches including radiotherapy,salvage chemotherapy and secondlook surgery are important for better local control.
文摘Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed.And a 4-year-old boy diagnosed with EST in our hospital was reported.The clinical manifestations,therapy,pathologic features,and prognosis of these cases were analyzed.Results Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review,including six cases searched from the PubMed and one case from our hospital.Six patients were boy and one patient’s gender was not available from the report.Ages ranged from 1 to 5 years(mean 3.14 years).The mean tumor size in our cohort was 4.4 cm.Six cases came from East Asia.Schiller-Duval bodies were found in all seven neoplasms.All tumors were positive for alpha-fetoprotein.The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy.The mean follow-up time was 24.4 months(range 5-52 months).Six tumors were totally removed,and four of them recurred.Three cases died including one whose tumor was partially removed.Conclusions The serum alpha-fetoprotein level is well correlated with the severity of the tumor.A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa.The prognosis of extragonadal intracranial EST is poor.
