摘要:目的:总结十二指肠间质瘤的诊断及外科手术体会。方法:回顾分析1999年~2008年收治的25例十二指肠间质瘤患者的临床资料。结果:临床表现最多见为黑便(14/25)，其次为右上腹不适(11/25)，腹块被(2/25)，无明显症状者(2/25)。术前诊断采用上消化道钡餐造影、CT、B超、胃镜或十二指肠镜、超声内镜检查。25例均手术治疗,其中胰十二指肠切除6例,局部切除18例,组织活检术+胃肠吻合1例。术后随访5~96个月,1、3、5年生存率为95.4%、85.5%和67.3%。结论:综合CT、胃肠道钡餐造影、消化内镜可使大部分十二指肠间质瘤术前得到确诊。手术方式依据肿瘤部位、大小而定,局部切除应选择正确重建方式。Abstract: Objective: To investigate the diagnosis and surgery treatment of duodenal gastrointestinal stromal tumors(GIST).Methods: The clinical data of 25 patients with GIST from 1999 to 2008 were analyzed retrospectively.Results: The most common symptoms of duodenal GIST were melena(14/25), as well as abdominal pain(11/25),abdominal mass, absence of symptoms(2/25). We performed the diagnosis by upper gastrointestinal radiography, gastroscopy, endoscopic ultrasonography and CT scan. All the 25 patients underwent surgical resection, of which 6 with pancreaticoduodenectomy, 18 with local resection, 1 with tissue biopsy and stomach intestinal anastomosis. With 5 to 96 months followup after operation, 1, 3 and 5year survival rates were 95.4%, 85.5% and 67.3%. Conclusion: Preoperative diagnosis of most of GIST was dependent on CT scan, upper gastrointestinal radiography and gastroscopy. The choices of surgical procedures are mainly determined by the location and size of the tumors, local excision should choose the correct way to rebulid alimentary tract.
ObjectiveTo summarize current treatment methods and research advances of liver metastasis in patients with gastrointestinal stromal tumor (GIST).MethodThe related literatures about treatment of liver metastasis in patients with GIST were collected and reviewed.ResultsGIST often occurred liver metastasis, which seriously affected the prognosis of patients. In the era of tyrosine kinase inhibitors (TKI) treatment, radical resection combined with TKI was the first choice. In addition, radiofrequency ablation and interventional therapy could be selected according to the patient’s condition.ConclusionsComplete resection of tumor and TKI treatment can improve the prognosis and survival rate of GIST patients with liver metastasis. GIST patients with liver metastasis need multi-disciplinary and multi-mode combined treatment.
ObjectiveTo investigate the current status and hotspots in researches of gastrointestinal stromal tumor.MethodsThe literatures related to gastrointestinal stromal tumor and published from 2000 to 2019 were extracted from Web of Science Core Collection. Visualization analysis was conducted by utilizing CiteSpace and VOSviewer software based on bibliometrics methods.ResultsA total of 3 226 articles and reviews were retrieved. The number of publications increased from 2000 to 2019, from the initial 34 publications gradually increased to 241 in 2019, and the maximum number of publications was 265 in 2015. The most productive five countries in terms of publication number were: 907 in the United States, 540 in China, 522 in Japan, 219 in Germany, and 214 in Italy. Co-occurrence networks of keywords were divided into three clusters: targeted therapy, clinical management, and pathogenesis research. Co-citation analysis of literatures contained 9 clusters, and cluster #0 prognosis was the latest cluster, indicating the research frontier and tendency.ConclusionsThe United States and its domestic institutes are the pioneers. Targeted therapy, clinical management, and pathogenesis researches are the main research filed. Prognosis research may be the new research tendency, and clinical and basic researches are still hotspots in medical research.
ObjectiveTo explore the clinical characteristics, diagnosis, and treatment norms of gastrointestinal stromal tumor, so as to deepen clinicians’ understanding of the gastrointestinal stromal tumor and avoid misdiagnosis.MethodsThe clinical data of a patient with gastrointestinal stromal tumor who treated in March 2019 in The First Hospital of Kunming was retrospectively analyzed, and determining the methods of the treatment through MDT mode.ResultsThis patient was generally in good condition. After MDT discussions among the imaging department, cardiothoracic surgery department, oncology department, and anesthesia department, it was considered that surgical treatment was the best treatment scheme. The operation time was 120 min, intraoperative bleeding was about 100 mL, and no blood transfusion was performed. No bleeding, abdominal infection, gastroesophageal reflux, and other complications occurred after the operation, and the patient was discharged successfully on the 10th day after the operation. Postoperative treatment was assisted by imatinib. Follow-up was conducted for more than 4 months, with no obvious complication occurred after discharge, so continued to follow-up.ConclusionGastrointestinal stromal tumor can be diagnosed and treated by multidisciplinary approach, and surgical resection is still the most important and effective treatment.
Objective To analyze features of color Doppler ultrasonography in gastrointestinal stromal tumors. Method The ultrasound images of gastrointestinal stromal tumors (51 cases) and gastrointestinal cancers (59 cases) confirmed by operation and pathology were compared and analyzed. Results The gastric stromal tumor mainly occurred at the bottom of the stomach and the body of the stomach (17 cases), the intestinal stromal tumor mainly occurred at the small intestine (24 cases). The gastric cancer mainly occurred at the gastric antrum (18 cases), the intestinal cancer all occurred at the colon (20 cases) and rectum (12 cases). Compared with the gastrointestinal cancers, the gastrointestinal cavity was not surrounded by tumor, the peripheral boundary was clear, the morphology was more regular, the internal echo was uneven, and there was no peripheral lymph node metastasis in the gastrointestinal stromal tumors, the differences were statistically significant (P<0.05). There were no significant differences in the degree of blood flow and tumor diameter between the gastrointestinal stromal tumors and the gastrointestinal cancers (P>0.05), but the blood flow of the intestinal stromal tumor was significantly more abundant as compared with the intestinal cancer (P<0.05). Conclusion Color Doppler ultrasonography, as a simple and rapid method, has a certain diagnostic value for differentiation of gastrointestinal stromal tumors and gastrointestinal cancers.
Objective To summarize progress on diagnosis and treatment of advanced gastrointestinal stromal tumor (GIST). Method Through the retrieval of relevant literatures, the advances in the diagnosis and treatment of advanced GIST in recent years were reviewed. Results The diagnosis of advanced GIST mainly depends on imaging examination such as CT, MRI and endoscopy or endoscopic ultrasound. The diagnosis can be confirmed by needle biopsy for advanced GIST patients considering preoperative imatinib treatment. At present, the imatinib is the first-line therapy for patients with advanced GIST, followed by sunitinib and other novel targeted drugs. A multidisciplinary treatment strategy that included targeted therapeutic agents, combining with surgical resection, radiofrequency ablation and embolism chemotherapy have brought dramatic clinical benefit for advanced GIST. Conclusions GIST is easy to metastasis, clinicians should ensure early diagnosis and early treatment. In course of imatinib treatment, an individualized therapeutic regimen should be applied to treat advanced GIST based on specific situation of patients.
ObjectiveTo analyze the relevant risk factors affecting postoperative relapse-free survival (RFS) in the primary gastrointestinal stromal tumors (GIST) and develop a Nomogram predictive model of postoperative RFS for the GIST patients. MethodsThe patients diagnosed with GIST by postoperative pathology from January 2011 to December 2020 at the First Hospital of Lanzhou University and Gansu Provincial People’s Hospital were collected, and then were randomly divided into a training set and a validation set at a ratio of 7∶3 using R software function. The univariate and multivariate Cox regression analysis were used to identify the risk factors affecting the RFS for the GIST patients after surgery, and then based on this, the Nomogram predictive model was constructed to predict the probability of RFS at 3- and 5-year after surgery for the patients with GIST. The effectiveness of the Nomogram was evaluated using the area under the receiver operating characteristic curve (AUC), consistency index (C-index), and calibration curve, and the clinical utility of the Nomogram and the modified National Institutes of Health (M-NIH) classification standard was evaluated using the decision curve analysis (DCA). ResultsA total of 454 patients were included, including 317 in the training set and 137 in the validation set. The results of multivariate Cox regression analysis showed that the tumor location, tumor size, differentiation degree, American Joint Committee onCancer TNM stage, mitotic rate, CD34 expression, treatment method, number of lymph node detection, and targeted drug treatment time were the influencing factors of postoperative RFS for the GIST patients (P<0.05). The Nomogram predictive model was constructed based on the influencing factors. The C-index of the Nomogram in the training set and validation set were 0.731 [95%CI (0.679, 0.783)] and 0.685 [95%CI (0.647, 0.722)], respectively. The AUC (95%CI) of distinguishing the RFS at 3- and 5-year after surgery were 0.764 (0.681, 0.846) and 0.724 (0.661, 0.787) in the training set and 0.749 (0.625, 0.872) and 0.739 (0.647, 0.832) in the validation set, respectively. The calibration curve results showed that a good consistency of the 3-year and 5-year recurrence free survival rates between the predicted results and the actual results in the training set, while which was slightly poor in the validation set. There was a higher net benefit for the 3-year recurrence free survival rate after GIST surgery when the threshold probability range was 0.19 to 0.57. When the threshold probability range was 0.44 to 0.83, there was a higher net benefit for the 5-year recurrence free survival rate after GIST surgery. And within the threshold probability ranges, the net benefit of the Nomogram was better than the M-NIH classification system at the corresponding threshold probability. ConclusionsThe results of this study suggest that the patients with GIST located in the other sites (mainly including the esophagus, duodenum, and retroperitoneum), with tumor size greater than 5 cm, poor or undifferentiated differentiation, mitotic rate lower than 5/50 HPF, negative CD34 expression, ablation treatment, number of lymph nodes detected more than 4, and targeted drug treatment time less than 3 months need to closely pay attentions to the postoperative recurrence. The discrimination and clinical applicability of the Nomogram predictive model are good.
Objective To detective KRAS and BRAF mutations in gastrointestinal stromal tumors (GISTs) and explore its significance in resistance of imatinib treatment. Methods Three hundred and eighty-one c-kit/PDGFRA mutation samples, 119 c-kit/PDGFRA wild type samples, and 19 pairs of samples before and after imatinib resistance from 519 patients with GIST were enrolled in this study. Polymerase chain reaction was used to detect KRAS exon 2 and BRAF exon 15 mutations. The survival data were evaluated in patients with KRAS or BRAF mutation. Results KRAS mutation was found in 2 cases (1.7%) of c-kit /PDGFRA wild type GISTs, the type of KRAS mutation was G12D and G12C, respectively. BRAFV600E mutation was found in 2 cases (1.7%) of wild type GISTs. No KRAS and BRAF mutations were found in the patients with the c-kit/PDGFRA mutation GISTs and pairs of GISTs before and after imatinib resistance. Two patients with KRAS mutation showed shorter progression free survivals for imatinib treatment. Two patients with BRAF mutation had longer recurrence free survivals. Conclusions Low frequency of KRAS or BRAF mutation only happens in wild type GISTs. KRAS mutation might be related to imatinib primary resistance, but not to secondary resistance.
Carney triad is a rare tumor syndrome with few reports. This case showed the enhanced CT and MRI images of a rare young woman patient with Carney triad (containing gastric stromal tumor, renal cell carcinoma, adrenal pheochromocytoma, and pulmonary chondrosarcoma), which is intended to provide a reference for clinical diagnosis and differential diagnosis. This case reminds the radiologists and clinicians that the patients with a history of primary gastrointestinal stromal tumor and neoplastic lesions occurring at specific sites (pulmonary chondrosarcoma, adrenal pheochromocytoma, renal cell carcinoma, etc.) need to be alerted to the possibility of combining with Carney triad.
ObjectiveThis review has summarized in detail the advances in computed tomography (CT) and magnetic resonance imaging (MRI) imaging in evaluating the efficacy of targeted therapy for gastrointestinal stromal tumor (GIST).MethodsTo summarize the image-related guidelines, consensus, international conference reports, and relevant knowledge of clinical research on the evaluation of the efficacy of GIST targeted therapy in recent years.ResultsThe CT and MRI manifestation after targeted treatment of GIST was closely related to pathological changes, including necrosis, cystic degeneration, and bleeding. CT was the preferred imaging method. Functional magnetic resonance imaging, such as diffusion weighted imaging (DWI), had made some progress. The main criteria for evaluating the efficacy of GIST targeted therapy were RECIST 1.1 and Choi criteria.ConclusionCT and MRI play an important role in evaluating the efficacy of targeted therapy for GIST.