Gastrointestinal Stromal Tumors
Gastrointestinal stromal tumors (GISTs) are a type of soft-tissue sarcoma (cancers of the muscles and other supporting tissues of the body). They may be found anywhere in or near the gastrointestinal tract; most commonly the stomach (50-60%) and small bowel (25-30%). Whilst they can occur at an age most people diagnosed with GIST are over 50.Information for Patients and the Public
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Gastrointestinal System Cancers
Soft Tissue Sarcoma
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MeSH term: Gastrointestinal Stromal Tumors
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A Review of Sleeve Gastrectomy Specimen Histopathology.
Am Surg. 2016; 82(11):1101-1104 [PubMed] Related Publications
The cut-off value of tumor size and appropriate timing of follow-up for management of minimal EUS-suspected gastric gastrointestinal stromal tumors.
BMC Gastroenterol. 2017; 17(1):8 [PubMed] Free Access to Full Article Related Publications
METHODS: A single-institution retrospective study was performed. 69 patients with EUS-suspected gastric GISTs were studied from November 2008 to March 2015. 69 patients with minimal gastric GISTs ≤2 cm diagnosed by EUS were followed for a mean period of 29 months (range, 12 to 70). An at least 20% increase of the maximal diameter of the tumors was set as a significant change.
RESULTS: During follow-up, Of the 69 minimal EUS-suspected GISTs, 16 (23.2%) showed significant changes in size. 11 out of 69 GISTs (15.9%), 6 out of 43 GISTs (14.0%), 7 out of 30 GISTs (23.3%) showed significant changes in size, at 1 year, 2 years, and more than 3 years respectively. The receiver operating characteristic curve analysis showed that the tumor size cut-off was 9.5 mm. Only 4.7 and 3.7% of gastric EUS-suspected GISTs of <9.5 mm in size showed significant changes at 1 year and 2 years, while 9.5% at more than 3 years. 34.6, 31.3 and 55.6% of gastric EUS-suspected GISTs of ≥ 9.5 mm in size showed significant changes at 1 year, 2 years and more than 3 years.
CONCLUSIONS: Minimal EUS-suspected GISTs, larger than 9.5 mm may be associated with significant progression. The patients with a ≥ 9.5 mm GIST should have a EUS 6-12months, while <9.5 mm GIST may have a EUS extended to every 2-3 years.
Gastrointestinal Stromal Tumor Showing Intense Tracer Uptake on PSMA PET/CT.
Clin Nucl Med. 2017; 42(3):200-202 [PubMed] Related Publications
Serosal and muscular layers incision technique in laparoscopic surgery for gastric gastrointestinal stromal tumors.
Asian J Endosc Surg. 2017; 10(1):92-95 [PubMed] Related Publications
MATERIALS AND SURGICAL TECHNIQUE: SAMIT involves resection of the mucosal and submucosal layers and then an incision in serosal and muscular layers around the tumor. SAMIT is simple and does not require special devices. The data of 13 patients who underwent laparoscopic wedge resection with SAMIT for primary gastric gastrointestinal stromal tumors were reviewed. No intraoperative complications were observed, and postoperative stenosis occurred in only one case of a middle stomach lesion. Adequate oncological resection was performed in all cases.
DISCUSSION: Laparoscopic wedge resection with SAMIT is technically and oncologically safe. It is useful for treating gastric gastrointestinal stromal tumors, including those close to the esophagogastric junction or pyloric ring.
Inactivating Mutations of RB1 and TP53 Correlate With Sarcomatous Histomorphology and Metastasis/Recurrence in Gastrointestinal Stromal Tumors.
Am J Clin Pathol. 2016; 146(6):718-726 [PubMed] Related Publications
METHODS: A cohort of 83 primary untreated GISTs was analyzed for mutations in TP53, RB1, and CDKN2A by massive parallel sequencing. Tumors with mutations in TP53 and RB1 were analyzed by fluorescence in situ hybridization for the corresponding gene loci.
RESULTS: Two GISTs harbored inactivating mutations in RB1, and two other GISTs displayed inactivating mutations in TP53 All four tumors were KIT mutant high-risk tumors with highly cellular sarcomatous histomorphology and variable combinations of plump spindle cells to epithelioid highly atypical cells and high mitotic activity. Three of these patients developed recurrent or metastatic disease, while the fourth patient showed tumor rupture intraoperatively. The combined overall frequency of TP53 and RB1 mutations was 13% considering high-risk or malignant GISTs.
CONCLUSIONS: TP53 and RB1 mutations seem to be restricted to high-risk/malignant GISTs and occur at an equal although relatively low frequency.
Gastrointestinal stromal tumours (GISTs): A descriptive study on 29 cases.
Arab J Gastroenterol. 2016; 17(4):185-187 [PubMed] Related Publications
PATIENTS AND METHODS: A retrospective descriptive study of 29 cases of GIST in gastroenterology and general surgery departments of Mohamed Tahar Maamouri Hospital (Nabeul, Tunisia) was conducted from January 2005 to March 2012.
RESULTS: Among the 29 patients, there were 18 males (62%) and 11 females (38%) with a median age of 63 years (range, 30-96years). The main symptoms were abdominal pain (40%) and weight loss (28%). The tumour was revealed by a complication in 5 cases (17%). Six patients (20.7%) had metastatic lesions. The most common sites were the stomach (41.4%) and the small intestine (17.3%). The median tumour size was 9.5cm (range, 1-30cm). Spindle cell tumours were the main histological type (62%). KIT was positive in the majority of cases (75%). Twenty-one patients with primary disease (72%) underwent a surgical resection. Imatinib was prescribed in 7 patients (24%). Sunitinib malate was indicated in 3 patients who had tumour progression under imatinib. Median survival was 17 months (range, 1-69months). Ten patients died.
CONCLUSION: The management of GISTs has considerably evolved during the last years. Surgical resection, which remains the mainstay of treatment, was indicated in the majority of patients. Imatinib treatment has not improved overall survival in metastatic and/or inoperable cases.
A lower dosage of imatinib in patients with gastrointestinal stromal tumors with toxicity of the treatment.
Medicine (Baltimore). 2016; 95(49):e5488 [PubMed] Free Access to Full Article Related Publications
Clinical Significance of the Glasgow Prognostic Score in Patients with Gastrointestinal Stromal Tumors.
Anticancer Res. 2016; 36(12):6687-6690 [PubMed] Related Publications
PATIENTS AND METHODS: Twenty-nine patients with gastric GISTs undergoing gastrectomy were retrospectively reviewed. Patients were classified based on GPS criteria as follows: GPS of 2: elevated C-reactive protein (>1.0 mg/dl) and hypoalbuminemia (<3.5 g/dl), GPS of 1: one of these hematological abnormalities, and GPS of 0: neither elevated CRP nor hypoalbuminemia.
RESULTS: The National Institutes of Health (NIH) classification for a risk stratification demonstrated that 3 (10.3%), 15 (51.7%), 5 (17.2%), and 6 (20.7%) patients were at very low, low, intermediate, and high risk, respectively, of disease recurrence. GPS criteria classified 24 (82.8%), five (17.2%), and no (0%) patients into GPS of 0, 1, and 2, respectively. Postoperative recurrence was identified in five patients (17.2%). Disease recurrence correlated with a risk stratification based on the NIH or GPS classification (p=0.004 and p=0.024, respectively).
CONCLUSION: The GPS, as well as NIH classification, is a promising blood predictor of disease recurrence in patients with resectable gastric GISTs.
SEOM Clinical Guideline for gastrointestinal sarcomas (GIST) (2016).
Clin Transl Oncol. 2016; 18(12):1221-1228 [PubMed] Free Access to Full Article Related Publications
Detection of mutations in the BRAF gene in patients with KIT and PDGFRA wild-type gastrointestinal stromal tumors.
Virchows Arch. 2017; 470(1):29-36 [PubMed] Related Publications
Diagnosis and treatment of gastrointestinal stromal tumor extending to prostate: A case report and literature review.
Medicine (Baltimore). 2016; 95(46):e5439 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS: The patient complained about the retention of urinary.
INTERVENTIONS: radical prostatectomy and the imatinib therapy.
OUTCOMES: No recurrence and metastasis have been found during a 14-month follow-up.
LESSONS: comprehensive treatment is necessary for the GIST treatment. Furthermore, we summarize a review of the literature of GIST occurring in the prostate gland treated by different methods and 4 kinds of rare diseases in prostate.
Clinicopathological features and prognosis of coexistence of gastric gastrointestinal stromal tumor and gastric cancer.
Medicine (Baltimore). 2016; 95(45):e5373 [PubMed] Free Access to Full Article Related Publications
New Drug and Possible New Toxicity - Squamous Cell Carcinoma Following Imatinib in Patients with Gastrointestinal Stromal Tumors.
Anticancer Res. 2016; 36(11):6201-6204 [PubMed] Related Publications
CASE SERIES: Herein, we report a case series of cutaneous squamous cell carcinoma (SCC) occurring secondary to imatinib in two patients treated for GISTs. Both patients were successfully managed with surgical resection of SCC and the discontinuation of the drug. Furthermore, we undertook a comprehensive literature review on this association. Few cases of cutaneous SCC secondary to imatinib therapy were reported in patients with chronic myeloid leukemia. However, there was no clinical evidence on causation of imatinib-associated SCC in patients with GIST.
CONCLUSION: To our knowledge, the present report is the first to describe imatinib-related SCC in patients undergoing treatment for GISTs. This implicates that safety and long-term tolerability of imatinib in patients with GISTs warrant rigorous testing and close monitoring.
Imatinib-induced Gastrointestinal Vascular Ectasia in a Patient with Advanced GIST: Case Report and Literature Review.
Anticancer Res. 2016; 36(11):6151-6154 [PubMed] Related Publications
CASE REPORT: We present one patient with GIVE complicating imatinib therapy with a literature review of this rare side-effect.
RESULTS: A 68-year-old woman was diagnosed with advanced GIST, wild-type CKIT. After 3 months of treatment with imatinib, she had partial response. However, she was diagnosed with GAVE and, later, also with GIVE. During her 3-year imatinib treatment, she suffered from severe anemia and required blood transfusions. Conservative treatments were not helpful and the ectatic lesions resolved only with cessation of imatinib.
CONCLUSION: This confirms a causal relationship between GIVE and imatinib. GIVE and GAVE should be considered possible causes of anemia and upper gastrointestinal bleeding in patients receiving imatinib therapy.
Role of upfront surgery for recurrent gastrointestinal stromal tumours.
ANZ J Surg. 2016; 86(11):910-915 [PubMed] Related Publications
METHODS: Data were retrospectively collected from patients with recurrent GIST treated at Singapore General Hospital and National Cancer Centre Singapore over a 12-year period. Our primary end points were disease-free and overall survival (OS).
RESULTS: A total of 186 patients underwent curative surgery for GIST between January 2000 and June 2012. Fifty-six (30%) patients experienced recurrence, of which 30 (54%) had resectable recurrent disease. Twenty-four patients underwent upfront surgery for their recurrence while the remaining six patients opted for non-surgical management. The median OS for all patients with recurrent GIST was 5.3 years (95% confidence interval (CI) 3.2-8.4). It was not reached for patients who underwent curative surgery for their recurrence, and was 3.9 years (95% CI 2.4-7.0) for patients who had palliative TKI and conservative management. There were significant differences in OS and disease-specific survival between patients who underwent curative surgery for recurrence compared with those who had not.
CONCLUSION: Our study shows that upfront surgery is a reasonable treatment strategy for selected patients with recurrent GIST.
Targeting Gastrointestinal Stromal Tumor with (68)Ga-Labeled Peptides: An In Vitro Study on Gastrointestinal Stromal Tumor-Cell Lines.
Cancer Biother Radiopharm. 2016; 31(8):302-310 [PubMed] Related Publications
Synchronous Occurrence of Diffuse Large B-cell Lymphoma of the Duodenum and Gastrointestinal Stromal Tumor of the Ileum in a Patient with Immune Thrombocytopenic Purpura.
Intern Med. 2016; 55(20):2951-2956 [PubMed] Free Access to Full Article Related Publications
Cytologic and Immunohistochemical Evaluation of Low-Grade Spindle Cell Lesions of the Gastrointestinal Tract.
Arch Pathol Lab Med. 2016; 140(10):1038-44 [PubMed] Related Publications
Cost-Effectiveness Analysis of Tyrosine Kinase Inhibitors for Patients with Advanced Gastrointestinal Stromal Tumors.
Clin Drug Investig. 2017; 37(1):85-94 [PubMed] Related Publications
OBJECTIVE: The aim of this study was to compare four potential strategies (reflecting the potential daily practice), each including imatinib 400 mg/day, as first-line treatment: S1 (imatinib400/best supportive care [BSC]); S2 (imatinib400/imatinib800/BSC); S3 (imatinib400/sunitinib/BSC); and S4 (imatinib400/imatinib800/sunitinib/BSC).
METHODS: A Markov model was developed with a hypothetical cohort of patients and a lifetime horizon. Transition probabilities were estimated from the results of clinical trials. The analysis was performed from the French payer perspective, and only direct medical costs were included. Clinical and economic parameters were discounted, and the robustness of results was assessed.
RESULTS: The least costly and effective strategy was S1, at a cost of €65,744 for 32.9 life months (reference). S3 was the most cost-effective strategy, with an incremental cost-effectiveness ratio (ICER) of €48,277/life-year saved (LYS). S2 was dominated, and S4 yielded an ICER of €363,320/LYS compared with S3. Sensitivity analyses confirmed the robustness of these results; however, when taking into account a price reduction of 80 % for imatinib, S2 and S4 become the most cost-effective strategies.
CONCLUSION: Our approach is innovative to the extent that our analysis takes into account the sequential application of TKIs. The results suggest that the S1 strategy is the best cost-effective strategy, but a price reduction of imatinib impacts on the results. This approach must continue, including new drugs and their impact on the quality of life of patients with advanced GISTs.
Clinicopathologic features and prognosis of primary gastrointestinal stromal tumor patients under 35 years of age: A 10-year retrospective study.
J Surg Oncol. 2016; 114(8):977-981 [PubMed] Related Publications
METHODS: Clinicopathologic data from GIST patients under 35 years diagnosed at our hospital from January 2005 to December 2014 were retrospectively collected.
RESULTS: Thirty-one (5.3%, 31/585) patients were included; 17 (54.8%) were female. The most common presentation and primary tumor site were gastrointestinal bleeding (n = 18, 58.1%) and the small intestine (n = 13, 41.9%), respectively. Fifteen (48.4%) GISTs were classified as having a high relapse risk; two (6.4%), intermediate; nine (29.0%), low; and five (16.1%), very low. All patients underwent tumor resection. With a median follow-up of 51 months for 20 (64.5%) patients, 12 (60%) were given imatinib methylate as adjuvant therapy. One (5%) patient died of peritoneal GIST dissemination, four (20%) developed abdominal recurrences, two (10%) had hepatic metastasis, and thirteen (65%) were disease free. The 5-year disease-free survival rate was 51.2%.
CONCLUSIONS: GISTs rarely occur in young adults. The most common location is the small intestine. A slight female predominance was observed in the current study. Adjuvant therapy longer than the recommended duration may be beneficial for GISTs with a high relapse risk. Combined targeted therapy and surgery is appropriate for recurrent and metastatic GISTs in select patients. J. Surg. Oncol. 2016;114:977-981. © 2016 Wiley Periodicals, Inc.
Gastrointestinal stromal tumors (GISTs) and second malignancies: A novel "sentinel tumor"? A monoinstitutional, STROBE-compliant observational analysis.
Medicine (Baltimore). 2016; 95(38):e4718 [PubMed] Free Access to Full Article Related Publications
Key messages from the BFR14 trial of the French Sarcoma Group.
Future Oncol. 2017; 13(3):273-284 [PubMed] Related Publications
Endoscopic submucosal dissection for silent gastric Dieulafoy lesions mimicking gastrointestinal stromal tumors: Report of 7 cases-a case report series.
Medicine (Baltimore). 2016; 95(36):e4829 [PubMed] Free Access to Full Article Related Publications
METHODS: Data from the patients with gastric protrusion lesions who underwent ESD from September 2008 to April 2016 in General Hospital, Tianjin Medical University, China were recorded. Seven cases with pathological diagnosis of Dieulafoy lesion without bleeding were enrolled for further analysis.
RESULTS: A total of 7 patients (2 males and 5 females) with mean age of 57.7 ± 4.15 years were pathologically diagnosed as Dieulafoy lesion. Four of the lesions were located in gastric antrum, 2 in the fundus, and 1 in the body of stomach, respectively. The mean sizes of the Dieulafoy lesions under white light endoscopy and endoscopic ultrasonography (EUS) were 1.06 ± 0.28 and 0.84 ± 0.29 cm. The origins of these lesions were submucosa (6/7, 85.7%) and muscularis propria (1/7, 14.3%). Three of them appeared with mixed echo under EUS, 3 with hypoechogenicity, and 1 with hyperechogenicity. En bloc complete resection was achieved in all the lesions by ESD with average time of 76.00 ± 16.86 minutes, and no intraoperative bleeding happened. In addition, all patients were followed up for 1 to 53 months, and no recurrence or long-term complications was observed.
CONCLUSION: Therefore, ESD can be an effective and safe treatment for silent gastric Dieulafoy lesions with clinical presentations of submucosal protrusion lesions mimicking GISTs.
Promising novel therapeutic approaches in the management of gastrointestinal stromal tumors.
Future Oncol. 2017; 13(2):185-194 [PubMed] Related Publications
Molecular subtypes of gastrointestinal stromal tumors and their prognostic and therapeutic implications.
Future Oncol. 2017; 13(1):93-107 [PubMed] Related Publications
Plexiform angiomyxoid myofibroblastic tumor of the stomach: A case report.
Diagn Cytopathol. 2017; 45(1):55-58 [PubMed] Related Publications
Management of Gastrointestinal Stromal Tumors.
Surg Clin North Am. 2016; 96(5):1059-75 [PubMed] Related Publications
Advanced gastrointestinal stromal tumors: 10-years experience from a tertiary care centre.
Trop Gastroenterol. 2015 Jul-Sep; 36(3):168-73 [PubMed] Related Publications
METHODS: Medical records of GIST patients seen between years 2002-2012 were retrieved from institute as well as database maintained by authors. Patient included in this analysis had metastatic disease and unresectable and/or residual disease after surgery.
RESULTS: During the study period 62 patients fulfilled the inclusion criteria but 6 were lost to follow up before treatment and hence 56 patients were analysed. Median age was 45.5 years (range 17-70 years) with a male female ratio of 2:1. Thirty eight (67%) patients had metastatic disease whereas 32% patients had unresectable or incompletely resected disease. The most common primary site was small intestine in 24 (42.8%) which was followed by stomach in 11 (19.6%) patients. The most common site of metastases was liver in 27 (48%) patients. Median tumor size was 12 cm (range 4-50 cm). Thirty two (57%) patients had mitotic counts of > 5/50 HPF. All patients received imatinib. The most common response seen with imatinib was stable disease achieved in 29 (52%) patients. Imatinib was well tolerated by all patients without any drug discontinuation. The 5-year EFS and OS were 35% and 49%, respectively at a median follow up of 55 months. None of the patient or tumor factors were found to have prognostic significance in univariate survival analysis.
CONCLUSIONS: This is a single center experience of advanced GIST patients where small intestine was found to be the commonest disease site with imatinib producing disease stabilization in more than half of patients. Even though the survival was comparable to published reports, the major limitation was lack of mutation analysis.
A massive bleeding from a gastrointestinal stromal tumor of a Meckel's diverticulum.
Srp Arh Celok Lek. 2016 Mar-Apr; 144(3-4):219-21 [PubMed] Related Publications
CASE OUTLINE: The article presents the case of a 44-year-old otherwise healthy man with anemia, who was diagnosed lower gastrointestinal bleeding. An abdominal CT scan revealed a clearly demarcated solid tumor in hypogastric region, measuring 65 x 45 mm. A laparotomy through lower midline incision was performed. A surgical resection of a lesion of a Meckel's diverticulum was carried out and a final diagnosis of gastrointestinal stromal tumor was made.The patient made an uneventful recovery.
CONCLUSION: The preoperative diagnosis of a complicated Meckel's diverticulum may be challenging. CT is usually an adequate method to diagnose tumors arising from Meckel's diverticulum.
Laparoscopic gastric wedge resection as the method of choice in the treatment of gastrointestinal stromal tumors--A case report.
Srp Arh Celok Lek. 2016 Mar-Apr; 144(3-4):211-4 [PubMed] Related Publications
CASE OUTLINE: A 74-year-old patient was admitted to hospital with signs of bleeding from the upper parts of the gastrointestinal tract. Urgent gastroscopy was performed and a subepithelial gastric lesion with bleeding ulceration was noted in the region of the fornix. A computed tomography scan of the abdomen showed a tumor in the fornix region with the dimensions of 48 x 32 mm, which was growing mostly intraluminally. After an adequate preoperative preparation the patient underwent a laparoscopic wedge resection of gastric fornix with intramural tumor lesion. The histopathological analysis of the specimen showed a well differentiated GIST (histological grade Gi), of the spindle cell type. Based on the immunohistochemical analysis of thespecimen it was concluded that the patient was in the IA stage of the disease with a low risk of malignant progression. In the population of patients with GIST, this is the most common group (43%), with low malignant potential, and relapses present in only 3.6% of cases.The patient started with oral food intake on the first postoperative day, the first bowel movement occurred 36 hours after surgery, and the patient was released from hospital on the fourth postoperative day.
CONCLUSION: Based on the aforementioned, we consider that the laparoscopic gastric wedge resection is a safe and efficient surgical procedure. This is primary therapy for most common group of patients with resectable gastric GIST.