| Objective:In order to provide a diagnostic criteria and a comprehensive treatment strategy,the clinicopathological features and treatments of gastrointestinal stromal tumor(GIST)and extra-gastrointestinal stromal tumor(EGIST)were investigated,and the relationship between the clinicopathological features and prognosis of GIST and EGIST were evaluated.Methods:315 cases of GIST and 33 cases of EGIST confirmed by pathology department of the Affiliated Hospital of Southwest Medical University from January 2011 to August 2018 were analyzed retrospectively.Various factors,such as clinical features,pathological features and treatment strategy,were collected.Subsequently,patients were followed up.The relationship between the clinicopathological features and prognosis were evaluated.Results: 1.Clinicopathological features and prognosis of GIST 1.1 Clinical features: This study of GIST included 141 cases(44.8%)of male and 174 cases(55.2%)of female,and the incidence rate of male was slightly lower than that of female.The average age was 56.28 ± 11.76 years old and onset age focused on 51-60 years old.The clinical symptoms were unspecific.The most common initial symptoms were abdominal pain of 115 cases(36.5%),abdominal discomfort of 85 cases(26.9%),abdominal mass of 66 cases(21.0%)and no obvious clinical symptoms of 26 cases(8.3%).Before surgery,271 cases(86.0%)were examined by CT,34 cases(10.8%)by endoscopy and 10 cases(3.2%)by endoscopic ultrasonography.The tumor locations commonly involved in the stomach of 200 cases(63.5%),in the jejunum and ileum of 82 cases(26.1%),rarely in duodenum of 12 cases(3.8%),colorectum of 13 cases(4.1%)and esophagus of 8 cases(2.5%).Tumor rupture was founded in 21 cases(6.7%),but not in 294 cases(93.3%).1.2 Pathological features: Tumor diameter of GIST ranged from 0.4 cm to 25.0 cm,with an average diameter of 5.75 ± 4.66 cm.Meanwhile,there were masses which less than 5 cm of 160 cases(50.8%),between 5 cm and 10 cm of 110 cases(34.9%),and more than 10 cm of 45 cases(14.3%).About 86.0%(271 cases)of masses were solid,2.2%(7 cases)of masses were cystic,and 11.8%(37 cases)of masses were cystic and solid.The main morphologic features were spindle cell type 309 cases(98.0%),epithelioid cell type 3 cases(1%),and mixed cell type 3 cases(1%).There were masses with mitoses ≤ 5 / 50 HPF of 252 cases(80.0%),mitoses with 6-10/ 50 HPF of 47 cases(14.9%),and mitoses > 10 / 50 HPF of 16 cases(5.1%).Tumor necrosis was found in 62 cases(19.7%),but not in 253 cases(80.3%).About 21 cases(8.9%)involved in invading the surrounding tissues and 287 cases(91.1%)of tumors did not involve in invading the surrounding tissues.In addition,there were 58 cases(18.4%)in extremely low risk group of,97cases(30.8%)in low risk group,58 cases(18.4%)in medium risk group and 102 cases(32.4%)in high risk group.The positive rate of CD117 was 92.1%(290 / 315),that of DOG-1 was 95.2%(300 / 315),that of CD34 was 88.6%(279 / 315).Besides,there were 285 cases(90.5%)in Ki-67 ≤ 10% group and 30 cases(9.5%)in Ki-67 > 10% group.1.3 Treatment: 312 patients(99.0%)received complete tumor resection and 3 patients(1.0%)received partial tumor resection due to large volume.Subsequently,145 patients received postoperative imatinib treatment.1.4 Prognosis: 258 patients of GIST were successfully followed up.57 patients of GIST were lost to follow-up.The follow-up rate was 81.9%.The mean survival time was 99.00 ± 2.86 months.The 1-,3-,5-,and 7-year overall survival(OS)rates were 98.8%,91.6%,81.2% and 79.6% respectively,and the 1-,3-,5-,and 7-year progression-free survival(PFS)rates were 98.1%,88.3%,74.6% and 73.0% respectively.OS was related to various factors including tumor location,tumor rupture,tumor diameter,solid tumor,mitoses,tumor necrosis,tumor invasion of surrounding tissues,NIH grade,Ki-67 positive percentage and postoperative imatinib treatment(P < 0.05).While,PFS was related to various factors including age,tumor location,tumor rupture,tumor diameter,solid tumor,mitoses,tumor necrosis,tumor invasion of surrounding tissues,NIH grade,Ki-67 positive percentage(P < 0.05).Tumor location,tumor rupture,solid tumor,mitoses and postoperative imatinib treatment were independent factors for OS(P < 0.05).Tumor location and postoperative imatinib treatment were independent factors for PFS(P < 0.05)2.Clinicopathological features and prognosis of EGIST 2.1 Clinical features: This study of EGIST included 11 cases(33.3%)of male and 22 cases(66.7%)of female,and the incidence rate of male was slightly lower than that of female.The average age was 53.18 ± 13.08 years old and onset age focused on 24-76 years old.The clinical symptoms were unspecific.The most common initial symptoms were abdominal pain of 13 cases(39.4%),abdominal discomfort of 5 cases(15.2%),abdominal mass of 8 cases(24.2%)and no obvious clinical symptoms of 6 cases(8.3%).Before surgery,31 cases(94.0%)were examined by CT,2 cases(6.0%)by ultrasonography.The tumors mainly occurred in 7 cases of omentum(21.2%),6 cases of mesentery(18.2%),4 cases of retroperitoneum(12.2%),5 cases of hepatogastric or splenogastric space(15.2%),4 cases of pancreas(12.1%),2 cases of liver(6.0%),3 cases of pelvis(9.1%)and 2 cases of right lower extremity(6.0%).Tumor rupture was founded in 21 cases(18.2%),but not in 21 cases(81.8%).2.2 Pathological features: Tumor diameter of EGIST ranged from 2.0 cm to 26.0 cm,with an average diameter of 10.72 ± 5.82 cm.Meanwhile,there were tumors which less than 5 cm of 3 cases(9.1%),between 5 cm and 10 cm of 17 cases(51.5%),and more than 10 cm of 13 cases(39.4%).About 63.6%(21 cases)of tumors were solid.All cases were spindle cell type in histological type.There were tumors with mitoses ≤ 5 / 50 HPF of 15 cases(45.5%),mitoses with 6 ~10/ 50 HPF of 10 cases(30.3%),and mitoses > 10 / 50 HPF of 8 cases(24.2%).Tumor necrosis was found in 13 cases(39.4%),but not in 20 cases(60.6%).About 1 case(3.0%)involved in invading the surrounding tissues and 32 cases(97.0%)of tumors did not involve in invading the surrounding tissues.In addition,there were 2 cases(6.1%)in low risk group,2 cases(6.1%)in medium risk group and 29 cases(87.9%)in high risk group.The positive rate of CD117 was 93.9%(31/33),that of DOG-1 was 93.9%(31/33),that of CD34 was 72.7%(24/33).Besides,there were 27 cases(81.8%)in Ki-67 ≤ 10% group and 6 cases(18.2%)in Ki-67 > 10% group.2.3 Treatment: All 33 patients received complete tumor resection.Subsequently,26 patients received postoperative imatinib treatment.2.4 Prognosis: 29 patients of GIST were successfully followed up.4 patients of GIST were lost to follow-up.The follow-up rate was 87.8%.The mean survival time was 61.0 ± 2.86 months.There were 13 patients died and 1 patient with metastasis.The 1-,3-,5-,and 7-year OS rates were 86.2%,70.4%,55.3% and 44.3% respectively,and the 1-,3-,5-,and 7-year PFS rates were 82.8%,59.2%,43.1% and 32.3% respectively.OS was related to various factors including tumor diameter,mitoses,tumor necrosis(P < 0.05).PFS was related to various factors including age,tumor diameter,mitoses,tumor necrosis(P < 0.05).Tumor diameter was an independent factor for OS(P < 0.05).3 Comparison of clinicopathological features and prognosis between GIST and EGIST 3.1 There were statistically significant differences in tumor rupture,solid tumor,mitoses,NIH grade,and postoperative recurrence and metastasis rate(P < 0.05).Compared with GIST patients,EGIST patients were more prone to tumor rupture,large tumor diameter,cystic change,high mitoses,high risk,and high rate of postoperative recurrence and metastasis rate.In addition,the positive rate of CD34 in EGIST was 72.7%,which was lower than that of GIST(88.6%),and the difference was statistically significant(P = 0.010).3.2 The 3-,5-,and 7-year overall and progression free survival rates of EGIST were significantly lower than those of GIST(P < 0.001).Conclusion: 1.The incidence rate of male in GIST and EGIST is slightly lower than that of females,and the clinical symptoms of them are unspecific.2.GIST commonly occurs in the stomach,followed in jejunum and ileum,rarely in the duodenum,colorectal and esophagus.EGIST commonly occurs in the omentum or mesentery,rarely in the pancreas,liver or right lower extremity.3.GIST and EGIST are mainly solid tumors,but EGIST tends to cystic changes.Spindle cell type is the most common histological morphology.4.The main treatment of GIST and GIST is complete tumor resection.Postoperative imatinib treatment could improve the prognosis of GIST,but has little effect on EGIST.5.In GIST,tumor location,tumor rupture,solid tumor,mitoses and postoperative imatinib treatment are independent factors for OS.Meanwhile,tumor location and postoperative imatinib treatment are independent factors for PFS.However,only tumor diameter is independent factor for OS in EGIST.6.In EGIST,tumor diameter is a unique independent factor for OS.There is no one independent factor for PFS.7.Compared with GIST,EGIST has larger tumor size,higher mitoses,higher risk,and higher rate of postoperative recurrence and metastasis.Meanwhile,the prognosis of EGIST is worse than that of GIST. |
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