Reappraisals of biological behaviors of PDGFRA mutant gastrointestinal stromal tumor
Objective To investigate the biological behavior spectrum of platelet-derived growth factor alpha receptor(PDGFRA)-mutant gastrointestinal stromal tumor(GIST),and to compare the clinical values of the Zhongshan method of benign and malignant evaluation with the modified National Institutes of Health(NIH)risk stratification.Methods A total of 119 cases of GIST with PDGFRA mutation who underwent surgical resection at Zhongshan Hospital,Fudan University from 2009 to 2020 were collected.The clinicopathological data,follow-up records,and subsequent treatment were reviewed and analyzed statistically.Results There were 79 males and 40 females.The patients ranged in age from 25 to 80 years,with a median age of 60 years.Among them,115 patients were followed up for 1-154 months,and 13 patients progressed to disease.The 5-year disease-free survival(DFS)and overall survival(OS)were 90.1％and 94.1％,respectively.According to the modified NIH risk stratification,8 cases,32 cases,38 cases,and 35 cases were very-low risk,low risk,intermediate risk,and high risk,and 5-year DFS were 100.0％,95.6％,94.3％,and 80.5％,respectively.There was no significant difference in prognosis among the non-high risk groups,only the difference between high risk and non-high risk groups was significant(P=0.029).However,the 5-year OS was 100.0％,100.0％,95.0％and 89.0％,and there was no difference(P=0.221).According to the benign and malignant evaluation Zhongshan method,43 cases were non-malignant(37.4％),56 cases were low-grade malignant(48.7％),9 cases were moderately malignant(7.8％),and 7 cases were highly malignant(6.1％).The 5-year DFS were 100.0％,91.7％,77.8％,38.1％,and the difference was significant(P＜0.001).The 5-year OS were 100.0％,97.5％,77.8％,66.7％,the difference was significant(P＜0.001).Conclusions GIST with PDGFRA gene mutation shows a broad range of biological behavior,ranging from benign to highly malignant.According to the Zhongshan method,non-malignant and low-grade malignant tumors are common,the prognosis after surgery is good,while the fewer medium-high malignant tumors showed poor prognosis after surgical resection.The overall biological behavior of this type of GIST is relatively inert,which is due to the low proportion of medium-high malignant GIST.The modified NIH risk stratification may not be effective in risk stratification for PDGFRA mutant GIST.
NETL
NSTL
万方数据
维普
