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Background: Small intestinal stromal tumors (SISTs) are a rare type of mesenchymal tumor. Gender is known to influence the incidence and prognosis of various tumors, but its role on the survival of SISTs at the population level remains unclear. Therefore, we aim to explore the relationship between gender and the prognosis of SISTs using the Surveillance, Epidemiology, and End Results (SEER) database., Methods: Data on SISTs patients from 2000 to 2019 were derived from the SEER database. Multiple imputation was used to address missing data. Kaplan-Meier analyses and Cox proportional hazard models were applied to evaluate the impact of demographic and clinical characteristics on overall survival (OS) and cancer-specific survival (CSS)., Results: A total of 3513 patients with SISTs were analyzed, including 1921 males and 1592 females. Kaplan-Meier analysis coupled with log-rank testing demonstrated a significantly higher mortality rate among male patients compared to females (P < 0.001). Notably, female patients exhibited superior OS (hazard ratio [HR] 0.808, 95% confidence interval [CI] 0.724-0.902, P < 0.001) and CSS (HR 0.801, 95% CI 0.692-0.927, P = 0.003) compared to male patients. While the mean 1-year CSS rates were comparable between genders (95.3% for males vs. 96.0% for females, P = 0.332), male patients consistently showed lower mean survival rates at 3-, 5-, and 10-year intervals. Surgical intervention significantly boosted 5-year OS and CSS rates in both male and female patients (P < 0.001). Multivariate Cox regression analysis identified age, sex, grade, TNM stage, surgery, and mitotic rate as independent risk factors for OS and CSS in patients with SISTs., Conclusions: Our findings suggest that male patients with SISTs have a higher risk of mortality compared to female patients, indicating that gender may serve as a predictive indicator for survival in this patient population., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Gastrointestinal Stromal Tumors (GISTs) are the most common mesenchymal tumors of the GI tract. SEER is an extensive cancer database which proves useful in analyzing population trends. This analysis investigated GIST outcomes between geriatric & non-geriatric patients., Methods: SEER*STAT 8.4.0.1 was used to extract relevant GIST data from 2000 to 2019. Geriatric age was defined as ≥70 years. Variables included age, sex, surgery, cancer-specific death, and overall survival. Statistical tests included univariate analysis using KM survival estimate (95% confidence interval) to calculate 5-year survival (5YS). Log-Rank tests determined statistical significance. Multivariable Cox's PH regression estimated the geriatric hazard death ratio adjusted for sex, stage, and surgery., Results: The number of patients included was 13,579, yielding overall 5YS of 68.6% (95% CI 67.7-69.5). Cancer-specific death was 39.11% in 2000 & 3.33% in 2019. Non-geriatric & geriatric patient data yielded 5YS of 77.4% (76.4%-78.3%) and 53.3% (51.7%-54.8%) respectively (p < 0.0001). For no surgery/surgery, younger patient data yielded 5YS of 48.7% (45.8%-51.4%) and 83.7% (82.7%-84.7%) respectively (p < 0.0001); geriatric data yielded 5YS of 29.3% (26.5%-32.1%) and 62.8% (60.8%-64.6%) respectively (p < 0.0001). Multivariable analysis yielded a geriatric hazard death of 2.56 (2.42-2.70) (p < 0.0001)., Conclusions: Cancer-specific death decreased since 2000, indicating an improvement in survival & treatment methods. Observed lower survival rates overall in the geriatric group. Surgery appeared to enhance survival rates in both groups, suggesting that surgery is an important factor in GIST survival regardless of age. Large prospective studies will help define clinical management for geriatric patients., (© 2024 International Society of Surgery/Société Internationale de Chirurgie (ISS/SIC).)

Objective: To investigate the feasibility of multi-slice spiral CT(MSCT) imaging features of gastric stromal tumor (GST) in predicting pathological NIH risk classification, providing imaging basis for patients with GST before treatment. Methods: The clinical and CT imaging data of 504 patients(506 GST lesions), 259males and 245 females, aged from 13 to 85(60±11) years, with GST confirmed by surgery and pathology collected in the Zhongshan Hospital Affiliated to Fudan University and the Affiliated TCM Hospital of Southwest Medical University. According to pathological NIH risk classification, 506 lesions were divided into low risk group (very low and low risk degree, 277 lesions) and high risk group (medium and high risk degree, 229 lesions).Clinical data and imaging characteristics were compared between two groups. Multivariate logistic regression analysis was performed to screen out independent risk factors for statistically significant imaging indicators. Receiver operating curve (ROC) was used to evaluate the predictive value of tumor length for risk classification. Resulst: Between low risk group and high risk group,there were significant differences in gender(male/female:131/146 vs 129/100), gastrointestinal bleeding(present/absent:39/238 vs 59/170), morphology(regular/Irregular:218/59 vs 95/134), calcification(present/absent:36/241 vs 53/176), degree of necrosis(0°/Ⅰ°/Ⅱ°/Ⅲ°:197/61/16/3 vs 58/98/32/41), ulceration(present/absent:32/245 vs 94/135), growth pattern(endophytic/exophytic/mixed:102/105/70 vs 44/98/87), tumor location(fundus/cardia/body/angle/antrum:98/7/135/12/25 vs 98/6/114/5/6), feeding artery(present/absent:32/245 vs 104/125), vascular enhancement(present/absent:19/258 vs 88/141), effusion of around the disease(present/absent:0/277 vs 13/216), positive sign of fat around the disease(present/absent:0/277 vs 30/199),maximum long diameter[2.82(2.04,3.80) cm vs 5.93(4.06,8.29) cm] and short diameter [2.31(1.60,2.88) cm vs 4.40(3.21,6.37) cm]of tumor(all P <0.05).The maximum long diameter of tumor ( OR =2.08,95% CI :1.35-3.20) and ulceration positive( OR =2.01,95% CI :1.03-3.92) were independent risk factors of risk classification(all P <0.05).Gastric antrum was used as the reference for tumor location, gastric fundus( OR =7.77,95% CI :2.00-30.24) and gastric body( OR= 3.93,95% CI :1.03-15.01) were independent risk factors of risk classification(all P <0.05).The area under curve(AUC) of the maximum long diameter of tumor for predicting risk classification was 0.87, and the optimal critical value, sensitivity and specificity were 4.98cm, 62.9% and 95.3% respectively. Conclusions: MSCT image features of GST had certain characteristics. MSCT has certain predictive value for pathological NIH risk classification of GST, which can provide certain imaging basis for patients before treatment.

Objective: This study aimed to compare the computed tomography (CT) features of gastric and small bowel gastrointestinal stromal tumors (GISTs) and further identify the predictors for risk stratification of them, respectively., Methods: According to the modified National Institutes of Health criteria, patients were classified into low-malignant potential group and high-malignant potential group. Two experienced radiologists reviewed the CT features including the difference of CT values between arterial phase and portal venous phase (PVPMAP) by consensus. The CT features of gastric and small bowel GISTs were compared, and the association of CT features with risk grades was analyzed, respectively. Determinant CT features were used to construct corresponding models., Results: Univariate analysis showed that small bowel GISTs tended to present with irregular contour, mixed growth pattern, ill-defined margin, severe necrosis, ulceration, tumor vessels, heterogeneous enhancement, larger size, and marked enhancement compared with gastric GISTs. According to multivariate analysis, tumor size (P < 0.001; odds ratio [OR], 3.279), necrosis (P = 0.008; OR, 2.104) and PVPMAP (P = 0.045; OR, 0.958) were the independent influencing factors for risk stratification of gastric GISTs. In terms of small bowel GISTs, the independent predictors were tumor size (P < 0.001; OR, 3.797) and ulceration (P = 0.031; OR, 4.027). Receiver operating characteristic curve indicated that the CT models for risk stratification of gastric and small bowel GISTs both achieved the best predictive performance., Conclusions: Computed tomography features of gastric and small bowel GISTs are different. Furthermore, the qualitative and quantitative CT features of GISTs may be favorable for preoperative risk stratification., Competing Interests: The authors declare no conflict of interest and source of funding., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: This study aimed to report the clinicopathological features, management and long-term outcomes of patients with gastrointestinal stromal tumours (GISTs) in Oman., Methods: This retrospective study was conducted on patients treated for GIST between January 2003 and December 2017 at three tertiary referral centres in Muscat, Oman. All patients with confirmed histopathological diagnoses of GIST and followed-up at the centres during this period were included. Relevant information was retrieved from hospital records until April 2019., Results: A total of 44 patients were included in the study. The median age was 55.5 years and 56.8% were female. The most common primary site of disease was the stomach (63.6%) followed by the jejunum/ileum (18.2%). Two patients (4.5%) had c-Kit -negative, discovered on GIST-1 -positive disease. A total of 24 patients (54.5%) presented with localised disease and eight (33.3%) were classified as being at high risk of relapse. Patients with metastatic disease received imatinib in a palliative setting, whereas those with completely resected disease in the intermediate and high-risk groups were treated with adjuvant imatinib. Of the six patients (13.6%) with progressive metastatic disease, of which four had mutations on exon 11 and one on exon 9, while one had wild-type disease. Overall, rates of progression-free survival and overall survival (OS) at 100 months were 77.4% and 80.4%, respectively. Rates of OS for patients with localised and metastatic disease were 89.9% and 80.2%, respectively., Conclusion: The presenting features and outcomes of patients with GISTs in Oman were comparable to those reported in the regional and international literature., Competing Interests: CONFLICT OF INTEREST The authors declare no conflicts of interest., (© Copyright 2021, Sultan Qaboos University Medical Journal, All Rights Reserved.)

Background: Although tumor size and mitotic rate are established prognostic factors for worse survival in patients undergoing surgical resection for gastric gastrointestinal stromal tumors, the impact of microscopic margins, or R1 resection, is not completely established., Methods: Patients who received no neoadjuvant therapy and underwent surgical resection for stage I to III gastric gastrointestinal stromal tumors were identified from the 2010 to 2013 National Cancer Database and divided into 2 cohorts, R0 and R1 resections. Cox proportional hazards ratio and Kaplan Meier survival estimates were utilized to analyze 5-y overall survival., Results: Of 2,084 patients, those with R1 resection (57, 2.7%) were more likely to have tumors >10 cm (28.1% vs 11.9%, odds ratio 3.51, P = .017) and stage III disease (26.3% vs 11.2%, odds ratio 2.26, P = .047). Although margin status was associated with higher risk tumors, it was not associated with receipt of adjuvant therapy. After multivariate Cox regression, R1 and R0 patients did not have a difference in 5-y overall survival (82.5% vs 88.6%, hazards ratio 1.26, P = .49). When stratified by stage of disease, there remained no difference in survival across all stages when comparing R1 and R0 patients., Conclusion: Positive microscopic margins are uncommon but do not appear to impact survival outcomes in patients with resected localized gastric gastrointestinal stromal tumors., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Importance: With the approval of avapritinib for adults with unresectable or metastatic gastrointestinal stromal tumors (GISTs) harboring a platelet-derived growth factor receptor alpha (PDGFRA) exon 18 variant, including PDGFRA D842V variants, and National Comprehensive Cancer Network guideline recommendations as an option for patients with GIST after third-line treatment, it is important to estimate the potential financial implications of avapritinib on a payer's budget., Objective: To estimate the budget impact associated with the introduction of avapritinib to a formulary for metastatic or unresectable GISTs in patients with a PDGFRA exon 18 variant or after 3 or more previous treatments from the perspective of a US health plan., Design, Setting, and Participants: For this economic evaluation, a 3-year budget impact model was developed in March 2020, incorporating costs for drug acquisition, testing, monitoring, adverse events, and postprogression treatment. The model assumed that avapritinib introduction would be associated with increased PDGFRA testing rates from the current 49% to 69%. The health plan population was assumed to be mixed 69% commercial, 22% Medicare, and 9% Medicaid. Base case assumptions included a GIST incidence rate of 9.6 diagnoses per million people, a metastatic PDGFRA exon 18 mutation rate of 1.9%, and progression rate from first-line to fourth-line treatment of 17%., Exposures: The model compared scenarios with and without avapritinib in a formulary., Main Outcomes and Measures: Annual, total, and per member per month (PMPM) budget impact., Results: In a hypothetical 1-million member plan, fewer than 0.1 new patients with a PDGFRA exon 18 variant per year and 1.2 patients receiving fourth-line therapy per year were eligible for treatment. With avapritinib available, the total increase in costs in year 3 for all eligible adult patients with a PDGFRA exon 18 variant was $46 875, or $0.004 PMPM. For patients undergoing fourth-line treatment, the total increase in costs in year 3 was $69 182, or $0.006 PMPM. The combined total budget impact in year 3 was $115 604, or $0.010 PMPM, including an offset of $3607 in postprogression costs avoided or delayed. The higher rates of molecular testing resulted in a minimal incremental testing cost of $453 in year 3., Conclusions and Relevance: These results suggest that adoption of avapritinib as a treatment option would have a minimal budget impact to a hypothetical US health plan. This would be primarily attributable to the small eligible patient population and cost offsets from reduced or delayed postprogression costs.

Background: The incidence pattern of gastric cancer by histological types across major race/ethnic groups is unknown., Methods: Age-standardized rates from 1992-2016 by race/ethnicity were calculated using data from Surveillance, Epidemiology, and End Results Program (SEER). Annual percent changes (APCs) in rates and corresponding 95% confidence intervals (CIs) were calculated and pairwise comparison of rates between race/ethnic groups was performed using the Joinpoint Regression Program. Calendar periods of incidence rates of gastric cardia and non-cardia cancer by histological types across race/ethnicity groups were shown by figures., Results: The White population has the highest incidence of gastric cardia adenocarcinoma and the incidence is keeping constant from 1992 through 2016 except the decreasing in the Asian population (AAPC = -1.4, 95%CI (-2.1, -0.8)). Although the incidence of non-cardia adenocarcinoma is decreasing in each group, the descending trend in the Asian population is the quickest (AAPC = -3.8, 95%CI (-4.0, -3.5)). Gastric carcinoids were observed to have statistically significant increasing trends in all race/ethnicity groups, especially in Hispanic women from 0.4 per 100,000 to 1.6 per 100,000 persons. The incidence of gastrointestinal stromal tumors (GISTs) is rising, with Non-Hispanic blacks having the highest incidence., Conclusion: This study demonstrated disparities in the incidence of gastric cancer by histological types among different race/ethnic groups. Further investigations are warranted to understand the changing incidence patterns by race/ethnicity.

Aim: To investigate the prognostic value of preoperative fibrinogen concentration (FIB) and D-dimer-fibrinogen ratio (DFR) in gastrointestinal stromal tumors (GISTs)., Methods: The purpose of this study was to retrospectively analyze 170 patients with GISTs who were admitted to our hospital from January 2010 to December 2015. The optimal cutoff values of related parameters were estimated by receiver operating characteristic (ROC) curve analysis. The recurrence free survival (RFS) rate was evaluated using Kaplan-Meier curves. Univariate analysis and multivariate Cox regression models were used to analyze the prognostic factors of GISTs. The relationship between the FIB, D-dimer, DFR, platelet count (PLT), and the clinicopathological features of GISTs was described by the chi-square test or nonparametric rank sum test (Mann-Whitney test)., Results: In ROC analysis, the optimal cutoff values of FIB, D-dimer, DFR, and PLT were 3.24 g/L, 1.24 mg/L, 0.354, and 197.5 (× 10 9 /L), respectively. Univariate analysis and the Kaplan-Meier survival curve showed that FIB, D-dimer, DFR, PLT, National Institutes of Health (NIH) risk category, tumor size, tumor location, and mitotic index were significantly relevant to the 3-year and 5-year survival rate of patients ( P < 0.05). Cox multivariate regression analysis illustrated that FIB ( RR : 0.108, 95%CI: 0.031-0.373), DFR ( RR : 0.319, 95%CI: 0.131-0.777), and NIH risk category ( RR : 0.166, 95%CI: 0.047-0.589) were independent prognostic factors of the RFS rate ( P < 0. 05). Moreover, FIB, D-dimer, DFR, and PLT were correlated with the clinical features of GISTs., Conclusion: FIB, D-dimer, DFR, and PLT are all related to the prognosis of GISTs. Moreover, FIB and DFR may be independent risk factors for predicting the prognosis of resectable GISTs., Competing Interests: Conflict-of-interest statement: There was no conflict of interest in the present study.

Background: The liver is the most common metastatic site in patients with gastrointestinal stromal tumors (GISTs). The purpose of this study is to identify the incidence and predictive factors associated with synchronous liver metastases among patients with GISTs., Methods: A retrospective review of the Surveillance Epidemiology and End Results (SEER) database was performed., Results: Overall, 2757 patients were identified, of which 276 (10%) had synchronous liver metastases. The two-year survival of patients with synchronous liver metastases was 31.9% overall and 37.1% after undergoing surgery with curative intent. Primary tumor size >5 cm (5-10 cm: OR 2.97, 95% CI: 1.03-8.55, p = 0.044, >10 cm: OR 5.59, 95% CI: 1.95-16.07, p = 0.001), presence of nodal metastases (OR 4.09, 95% CI: 2.01-8.33, p < 0.001) and mitotic count >5/50 HPF (OR 1.58, 95% CI: 1.01-2.47, p = 0.044) were associated with the presence of liver metastases., Conclusions: One out of ten patients with GISTs presents with hepatic metastases. Primary tumor size >5 cm, presence of nodal metastases and mitotic count >5/50 HPF are associated with a higher risk of synchronous hepatic metastases., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Importance: Patients with resectable gastrointestinal stromal tumors (GISTs) might not receive the recommended duration of adjuvant therapy if their risk of recurrence is underestimated, which can have an impact on their recurrence-free survival (RFS)., Objective: To determine the extent of physician underestimation of risk of recurrence after complete primary GIST resection, the impact of underestimation on planned adjuvant treatment duration, and the association among high-risk patients of planned adjuvant treatment duration and RFS., Design, Setting, and Participants: This was a retrospective observational medical record review reported by participating oncologists in 2013. US patients with complete primary GIST resection after 2010 were grouped as underestimated or not if their oncologists' charted risk assessments were lower than assessments based on the Revised National Institutes of Health Consensus Criteria or not. Patients were followed by general community oncologists until death or the end of follow-up., Main Outcomes and Measures: Fisher exact tests compared planned adjuvant treatment duration between groups. Cox proportional-hazards models estimated the impact of planned adjuvant treatment duration on RFS., Results: A total of 109 oncologists reported information on 506 patients with GIST after primary resection (65.8% were high-risk and 8.7% were intermediate-risk). Physicians underestimated risk for 190 patients (37.5%); 30.1% of tumors with an intermediate-level mitotic count (6-10 per 50 high-powered fields) and an intermediate tumor size (6-10 cm) were correctly recognized as high-risk, as were 7.5% of nongastric tumors with an intermediate-level mitotic count and a tumor size of 2 to 5 cm. A smaller proportion of high-risk patients in the underestimated vs not-underestimated groups had at least 3 years of planned adjuvant therapy (36.1% vs 65.9%; P < .001). Planned adjuvant treatment of at least 3 years vs less than 3 years among high-risk patients conferred a lower hazard of recurrence and/or death (adjusted hazard ratio, 0.29; P < .001; 95% CI, 0.14-0.59)., Conclusions and Relevance: Overall, physicians tended to underestimate the risk of recurrence for many patients with GIST, especially for patients with tumors of intermediate size, intermediate-level mitotic count, and nongastric location, which had an impact on planned adjuvant therapy duration. Patients with at least 3 years of planned adjuvant treatment had longer RFS. Improved education on postresection risk assessment and risk reduction is needed.

Background: Laparoscopic sleeve gastrectomy (SG) is a well-tolerated and effective procedure for sustained weight loss and amelioration of weight-related co-morbidities. Rarely, unexpected pathology may be identified intraoperatively, which may alter the surgical plan. Gastrointestinal stromal tumors (GISTs) are among the more frequently encountered tumors and pose a particular concern because of their malignant potential. We review our findings of incidental tumors encountered during 1415 consecutive SGs., Methods: Abnormal pathology records from all patients who underwent SG at our institution between 2009 and 2014 were reviewed. Patient demographic characteristics and clinical characteristics, tumor characteristics, including immunohistochemistry, operative course, and patient follow-up were reviewed., Results: There were 17 incidental gastric mesenchymal tumors identified (1.2%) in 1415 SG procedures. This included 12 GISTs (.8%), 2 schwannomas (.1%), and 3 leiomyomas (.3%). In the majority of cases (1210/1415), the gastric specimens were not reviewed by a pathologist because there were no gross abnormalities appreciated by the surgeon. The GISTs were between .3 and 2.9 cm, and all were low grade with negative margins. Patients with GISTs tended to be older (mean age 55±9.3 y) than the rest of the patients. There was no evidence of recurrence on follow-up., Conclusion: Incidental gastric mesenchymal tumors are rarely encountered during SG. The vast majority were GISTs with an incidence of .8% in this population. Concomitant SG and tumor resection were feasible, without compromising the objectives of each. Complete tumor excision is necessary for tumors>2 cm., (Copyright © 2015 American Society for Bariatric Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: Sparse information is available about GISTs in uncommon locations. Our large database analysis aims to determine the characteristics of GISTs in the esophagus, colon and rectum and compare to gastric GISTs., Methods: The Surveillance Epidemiology and End Results (SEER) database was queried from 1990 to 2009 using CS SCHEMA v0203. Characteristics of each location were compared to gastric GISTs., Results: 4411 GIST (29 esophageal, 2658 stomach, 1463 small intestine, 126 colonic, and 135 rectal) from 1990 to 2009 were identified. Univariate and multivariate predictors of worse disease specific survival in both the entire cohort and surgical resection group include older age, male gender, tumor size > 5 cm, no surgical intervention and anatomical location. Although less likely to undergo surgical resection, esophageal GIST (all patients and resected) had a comparable survival to gastric GIST. A higher proportion of colonic GISTs presented with distant disease and had a worse disease specific survival when compared to rectal GISTs., Conclusion: Our results show a rising incidence in GISTs and highlight the characteristics of GISTs based on anatomical location. In addition, this is the first study to demonstrate that colonic GISTs behave differently when compared to rectal GISTs and warrants further prospective evaluation., (© 2015 Wiley Periodicals, Inc.)

Introduction: Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract, which are characterized in the majority of cases by activating mutations in KIT and platelet-derived growth factor receptor alpha (PDGFRA). The introduction of tyrosine kinase inhibitors has revolutionized the management of patients with metastatic GIST. However, complete surgical resection remains the mainstay of management for those with localized disease. Recently, three large trials have confirmed the benefit of adjuvant imatinib therapy in patients who were at high risk of recurrence following complete resection. In this setting, it is critical that oncologists understand the various GIST risk assessment criteria and be able to apply these methods to accurately assess the risk of recurrence and the need for adjuvant imatinib therapy., Purpose: The aim of this review is to outline the risk stratification systems currently available to oncologists who are treating patients with GIST, so they can be optimally applied for clinical decision-making.

Background: Small-bowel cancer is uncommon and, accordingly, little is known about the epidemiology of this malignancy, especially by race and subtype., Objective: The objective of this analysis was to describe the distribution of small-bowel cancer in the United States by demographic, pathological, and clinical features., Design: This study was retrospective in design., Setting: Data from 26 population-based cancer registries in the United States from 1995 to 2008 were used., Patients: Patients diagnosed with small-bowel cancer (topography codes C17.0-17.3 and C17.8-17.9) were included., Main Outcome Measures: The primary outcomes measured were race- and histology-specific incidence (age-adjusted rate trends and age-specific rates) of small-bowel cancer., Results: A total of 56,223 men and women diagnosed with small-bowel cancer were identified. The overall age-adjusted incidence rates for small-bowel cancer were 26.1 in men and 17.7 in women. Neuroendocrine tumors were the most common histological types of small-bowel cancer in men and women, followed by carcinoma, lymphoma, and sarcoma. In comparison with whites, the rate of small-bowel cancer was 42% greater in black men, 46% greater in black women, 34% lower in Asian-Pacific Islander men, and 37% lower in Asian-Pacific Islander women. Rates of small-bowel cancer were 24% lower in Hispanic men and 15% lower in Hispanic women than rates in non-Hispanics. The excess of small-bowel cancer in blacks and the deficit in Asian-Pacific Islanders were attributable mainly to the incidence of adenocarcinoma and carcinoid tumors. The incidence of GI stromal tumor was significantly higher among Asian-Pacific Islanders., Conclusions: This is one of the largest studies of small-bowel cancer to date. These cancer registry data showed substantial racial and ethnic variation in the incidence of histological subtypes of small-bowel malignancy that suggest possible etiologic diversity and/or disparities in detection.

Gastrointestinal stromal tumors (GIST) are a unique class of mesenchymal tumors identified within the past decade. Intense molecular and genetic study has been used to characterize these tumors and develop treatment strategies. Although the mainstay of treatment remains surgical resection, therapy targeted at inhibiting tyrosine kinases has had dramatic results. Because of the rapid accumulation of information about the diagnosis and treatment of these tumors, the National Comprehensive Cancer Network convened a GIST task force to provide updated recommendations in 2010. As understanding of these tumors advances, rapid changes in recommendations will continue and should warrant regular updates in tumor management., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature., Methods: During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included., Results: There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma., Conclusions: Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors.

Background: Imatinib was approved in 2002 for unresectable and metastatic gastrointestinal stromal tumors. Our objective was to determine if the introduction of imatinib coincided with improved survival from metastatic gastrointestinal stromal tumor in the U.S. population and in specific socioeconomic groups., Methods: Query of the Surveillance, Epidemiology, and End Results registry identified 552 patients with metastatic gastrointestinal stromal tumor between 1995 and 2004. Year of diagnosis was categorized into two periods, 1995 to 2000 and 2001 to 2004, to account for the effect of imatinib. Kaplan-Meier and multivariate Cox regression analyses were used to examine differences in survival between periods and among socioeconomic groups., Results: Median survival increased from 12 to 33 months from 1995 to 2000 to 2001 to 2004 (P < 0.001); survival at 47 months increased from 21% to 41%, respectively (P < 0.001). Median survival times for White, Black, Hispanic, and Asian or Pacific Islander, and for low-, middle-, and high-income groups increased significantly in the era of imatinib (all P < 0.05). On multivariate analysis, Black race [hazard ratio, 1.96; 95% confidence interval (95% CI), 1.15-3.32; P = 0.013], Hispanic race (hazard ratio, 2.11; 95% CI, 1.14-3.88; P = 0.017), and low income (hazard ratio, 1.81; 95% CI, 1.13-2.89; P = 0.014) were associated with the poorest survival during the 1995 to 2000 period. During 2001 to 2004, these disparities in survival were no longer statistically apparent., Conclusions: Survival from metastatic gastrointestinal stromal tumor has improved significantly in the era of imatinib. This improvement has been uniform across all socioeconomic groups, with concomitant elimination of socioeconomic survival disparities potentially due to an assistance program intended to provide universal access to imatinib therapy.

Background: By reappraising the National Institutes of Health (NIH) consensus criteria, we worked on establishing a modified scheme to identify highly lethal gastrointestinal stromal tumors (GISTs), which have an imperative demand for sequencing analysis to assess the suitability of an adjuvant imatinib trial., Methods: Clinicopathologic features, including NIH and modified schemes, were retrospectively analyzed for 289 patients with localized GISTs. We combined the very low/low-risk GISTs into a single "risk level I" group (5 cm and >10/50 HPF, with the rest of high-risk GISTs defined as "risk level III.", Results: The cumulative 5-year rate of disease-specific survival (DSS) for all 289 patients was 82%, and the DSS rates for patients with GISTs classified as risk levels I to IV were 100%, 96%, 67%, and 25% at 5 years, respectively. The prognostic differences were striking between the risk level II and III groups (P < .0001) and between the risk level III and IV groups (P = .0002). The higher risk level of our scheme represented the strongest independent adverse factor (risk ratio [RR] = 11.299 for risk level III; RR = 33.815 for risk level IV; P < .0001), followed by mixed/epithelioid histology (RR = 2.837, P = .003) and older age (>or=70 years, RR = 1.955, P = .044)., Conclusions: Remarkable prognostic heterogeneity exists in the high-risk category of the NIH scheme, which is not as effective as the modified criteria in identifying highly lethal GISTs that we classified as risk level IV.

Background: Inactivation of p16(INK4A) promotes G1/S progression of cell cycle. Minichromosome maintenance protein-2 (Mcm2), a novel cell proliferation marker, is known to better correlate with clinical outcomes than Ki-67 in many carcinomas. Since gastrointestinal stromal tumors (GISTs) sometimes remains challenging in prognostication, we analyzed the utility of these three markers in GISTs., Methods: Immunohistochemistry was performed in tissue microarrays of 277 primary GISTs and correlated with NIH consensus criteria and clinical outcomes., Results: The increment of NIH risk levels significantly correlated with increasing labeling indices (LI) of both Ki-67 (P <.001) and Mcm2 (P <.001) and loss of p16(INK4A) expression (P <.035). However, the latter aberration did occur in 23% of very low/low-risk GISTs. The relationship between Mcm2 and Ki-67 LIs could be modeled as linear (P <.001, r = 0.697), while Mcm2 LI was considerably higher (P <.001) with a stepwise escalation related to risk levels. Ki-67 LI >5% (P <.0001) and Mcm2 LI >10% (P <.0001) were strongly predictive of inferior disease-specific survival (DSS), while aberrant loss of p16(INK4A) only reached a trend (P = .0954). In multivariate analyses, independent adverse factors of DSS were high-risk category (RR = 16.93, P <.0001), metastatic disease (RR = 4.12, P = .0015), Ki-67 LI >5% (RR = 3.55, P = .001), and presence of epithelioid histology (RR = 2.17, P = .0308)., Conclusions: Prognostic efficacy of NIH consensus criteria is substantiated. P16(INK4A) deregulation can occur early in GIST tumorigenesis and marginally correlates with patient survival. Despite Ki-67 LI being an independent prognosticator, simultaneous detection of Mcm2 is recommended as a prognostic adjunct of GISTs, given its better sensitivity and stepwise escalation with increasing risk levels.

Objectives: The epidemiology of gastrointestinal stromal tumor has not been well examined, and prior studies often provide conflicting results. We conducted the first population-based study to evaluate the incidence and survival of malignant gastrointestinal stromal tumor in the United States., Methods: We utilized the Surveillance, Epidemiology, and End Results registry from the National Cancer Institute to identify all cases of malignant gastrointestinal stromal tumor diagnosed from 1992 to 2000. The age-adjusted incidence rates and the survival rates were calculated. Cox proportional hazards models were used to examine the risk of mortality., Results: Between 1992 and 2000, there were 1,458 cases of diagnosed gastrointestinal stromal tumor. The age-adjusted yearly incidence rate was 0.68/100,000. The mean age at diagnosis was 63 yr. Fifty-four percent were men and 46% were women. The incidence rate was higher among men and among Blacks. Fifty-one percent of cases were in the stomach, 36% small intestine, 7% colon, 5% rectum, and 1% in the esophagus. Fifty-three percent of cases were staged as localized, 19% regional, 23% distant, and 5% unstaged. The 1- and 5-yr relative survival rates were 80% and 45%, respectively. The Cox analysis showed that older age, Black race, advanced stage, and receipt of therapy were independent predictors of mortality., Conclusions: Malignant gastrointestinal stromal tumors rare, but are more common in the older population, men, and Blacks. Risk factors for mortality include older age, Black race, advanced stage, and no surgical intervention.