BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from...BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from our institute.Then,the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease.Our cohort included two males and three females.The median age was 21-years-old(range:8-54-years-old).All the patients received surgical treatment.Gross total resection(GTR),radiotherapy,and chemotherapy were administered in 3 patients,4 patients,and 1 patient,respectively.After a median follow-up of 36 mo,tumor progression was noticed in 4 patients;and 3 patients died of the disease.Pooled data(n=14)contained 5 males and 9 females with a median age of 19 years.The log-rank tests showed that GTR(P=0.011)could prolong progression-free survival,and radiotherapy(P<0.001)resulted in longer overall survival.CONCLUSION Patients with PIASPS suffer from poor outcomes.Surgical treatment is the first choice,and GTR should be achieved when the tumor is feasible.Patients with PIASPS benefit from radiotherapy,which should be considered as a part of treatment therapies.展开更多
腺泡状软组织肉瘤(alveolar soft-part sarcoma,ASPS)是一种罕见的软组织肉瘤(soft tissue sarcoma,STS),占所有STS不到1%,远处转移率高且常发生于青少年[1-2],ASPS对于传统化疗疗效较差。研究发现,抗血管生成的酪氨酸激酶抑制剂(tyrosi...腺泡状软组织肉瘤(alveolar soft-part sarcoma,ASPS)是一种罕见的软组织肉瘤(soft tissue sarcoma,STS),占所有STS不到1%,远处转移率高且常发生于青少年[1-2],ASPS对于传统化疗疗效较差。研究发现,抗血管生成的酪氨酸激酶抑制剂(tyrosine kinase inhibitor,TKI)与程序性死亡受体1(programmed cell death protein 1,PD-1)/程序性死亡-配体1(programmed cell death ligand 1,PD-L1)抑制剂对STS均显示出活性[3-4],且PD-1/PD-L1单抗和TKI都在中国临床肿瘤学会(Chinese Society of Clinical Oncology,CSCO)STS诊疗指南范围内[5]。一些研究也证明了两者的联合对STS有更好的疗效[6-7]。展开更多
文摘BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from our institute.Then,the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease.Our cohort included two males and three females.The median age was 21-years-old(range:8-54-years-old).All the patients received surgical treatment.Gross total resection(GTR),radiotherapy,and chemotherapy were administered in 3 patients,4 patients,and 1 patient,respectively.After a median follow-up of 36 mo,tumor progression was noticed in 4 patients;and 3 patients died of the disease.Pooled data(n=14)contained 5 males and 9 females with a median age of 19 years.The log-rank tests showed that GTR(P=0.011)could prolong progression-free survival,and radiotherapy(P<0.001)resulted in longer overall survival.CONCLUSION Patients with PIASPS suffer from poor outcomes.Surgical treatment is the first choice,and GTR should be achieved when the tumor is feasible.Patients with PIASPS benefit from radiotherapy,which should be considered as a part of treatment therapies.
文摘腺泡状软组织肉瘤(alveolar soft-part sarcoma,ASPS)是一种罕见的软组织肉瘤(soft tissue sarcoma,STS),占所有STS不到1%,远处转移率高且常发生于青少年[1-2],ASPS对于传统化疗疗效较差。研究发现,抗血管生成的酪氨酸激酶抑制剂(tyrosine kinase inhibitor,TKI)与程序性死亡受体1(programmed cell death protein 1,PD-1)/程序性死亡-配体1(programmed cell death ligand 1,PD-L1)抑制剂对STS均显示出活性[3-4],且PD-1/PD-L1单抗和TKI都在中国临床肿瘤学会(Chinese Society of Clinical Oncology,CSCO)STS诊疗指南范围内[5]。一些研究也证明了两者的联合对STS有更好的疗效[6-7]。
