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Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
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作者 Jun-Yu Chen Bo Cen +4 位作者 Fei Hu Yong Qiu Guo-Min Xiao Jun-Ge Zhou fang-cheng zhang 《World Journal of Clinical Cases》 SCIE 2022年第1期296-303,共8页
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
关键词 Primary intracranial alveolar soft-part sarcoma Surgery Radiotherapy Progression-free survival Overall survival Case report
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