NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022

Mehren, Margaret von; Kane, John M.; Riedel, Richard F.; Sicklick, Jason K.; Pollack, Seth M.; Agulnik, Mark; Carr‐Ascher, Janai R.; Choy, Edwin; Dry, Sarah M.; Ganjoo, Kristen N.; Gonzalez, Ricardo J.; Holder, Ashley M.; Homsi, Jade; Keedy, Vicki L.; Kelly, Ciara M.; Kim, Edward; Liebner, David A.; McCarter, Martin D.; McGarry, Sean V.; Mesko, Nathan W.; Meyer, Christian F.; Pappo, Alberto S.; Parkes, Amanda; Petersen, Ivy A.; Poppe, Matthew M.; Schuetze, Scott M.; Shabason, Jacob E.; Spraker, Matthew B.; Zimel, Melissa N.; Bergman, Mary Anne; Sundar, Hema; Hang, Lisa E

doi:10.6004/jnccn.2022.0058

Cited by 51 publications

(54 citation statements)

References 76 publications

(101 reference statements)

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“…Individuals suspected to have GISTs may experience various signs and symptoms, such as early satiety, postprandial pain, abdominal distension, bleeding to the abdominal cavity with acute abdomen, digestive tract bleeding, or exhaustion due to anemia [3,7]. Although risk factors for the development of GISTs have been studied, no environmental factors were identified.…”

Section: Case Presentationmentioning

confidence: 99%

“…GISTs can occur at any age but peak in the sixth decade of life [2]. The estimated annual occurrence of GISTs in the United States is 0.68 to 0.78 per 100,000 individuals, with the stomach being the most common site, accounting for 60% of cases, and the small intestine being the second (20%-30%) [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…Early diagnosis and treatment of GISTs are essential for a good prognosis, as they can be aggressive and have the potential to metastasize to other organs. Surgical resection is the mainstay of treatment for localized GISTs, while imatinib mesylate, a tyrosine kinase inhibitor, is used for adjuvant therapy and for metastatic or unresectable diseases with significantly improved survival rates [3,4]. In addition to imaging studies, endoscopic techniques such as capsule endoscopy and balloon-assisted enteroscopy have been used.…”

Section: Introductionmentioning

confidence: 99%

“…GISTs are typically treated with surgery, and the prognosis is largely dependent on the tumor size, mitotic rate, and tumor location [3]. Adjuvant therapy with tyrosine kinase inhibitors, such as imatinib, may be considered for high-risk GISTs or for cases with residual disease after surgery [6].…”

Section: Introductionmentioning

confidence: 99%

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Jejunal Gastrointestinal Stromal Tumor: A Diagnostic Challenge

Miranda¹,

Trokhimtchouk²,

Flores³

et al. 2023

Cureus

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Gastrointestinal stromal tumors (GISTs) are a common type of soft tissue sarcoma that originates from the interstitial cells of Cajal in the gastrointestinal (GI) tract. These tumors usually affect people above 50 years of age and can be difficult to diagnose, as symptoms can be vague and nonspecific, with some patients remaining asymptomatic. Early diagnosis and treatment are crucial because GISTs can be aggressive and may metastasize.We present a case of a 74-year-old man who presented to our hospital with GI bleeding and anemia. Despite initial investigations, the source of bleeding was not identified until capsule endoscopy and then balloon enteroscopy revealed an ulcerated mass in the jejunum. The tumor was successfully removed using a minimally invasive laparoscopic approach, and the histopathologic report confirmed the diagnosis of GIST. The patient had an uneventful postoperative course. This case highlights the importance of considering GISTs in the differential diagnosis of obscure GI bleeding. A multidisciplinary approach is essential to ensure the best outcomes for these patients. Additionally, the use of minimally invasive surgery should be considered whenever possible to minimize postoperative complications and promote faster recovery.

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Section: Case Presentationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Jejunal Gastrointestinal Stromal Tumor: A Diagnostic Challenge

Miranda¹,

Trokhimtchouk²,

Flores³

et al. 2023

Cureus

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“…Unlike HP, the biological behavior of GIST is complex and demonstrates varying malignant potential, including recurrences and metastasis [16][17][18]. Surgery for GISTs was more focused on the risk class rather than just the size or location of the entities [18,19], and patients often choose regular followup visits or surgeries.…”

Section: Introductionmentioning

confidence: 99%

A CT-based nomogram established for differentiating heterotopic pancreas from gastrointestinal stromal tumor: compared with a machine-learning model

Feng

Chen

Wang

et al. 2023

Preprint

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Objective: To identify CT features and establish a nomogram, compared with a machine learning-based model for distinguishing heterotopic pancreas (HP) from gastrointestinal stromal tumor (GIST). Materials and methods: This retrospective study included 148 patients with pathologically confirmed HP (n = 48) and GIST (n = 100) in the stomach or small intestine that were less than 3 cm in size. Clinical information and CT characteristics were collected. A nomogram on account of lasso regression and multivariate logistic regression, and a RandomForest (RF) model based on significant variables in univariate analyses were established. Receiver operating characteristic (ROC) curve, mean area under the curve (AUC), calibration curve and decision curve analysis (DCA) were carried out to evaluate and compare the diagnostic ability of models. Results: The nomogram identified five CT features as independent predictors of HP diagnosis: age, location, LD/SD ratio, duct-like structure, and HU lesion / HU pancreas A. Five features were included in RF model and ranked according to their relevance to the differential diagnosis: LD/SD ratio, HU lesion / HU pancreas A, location, peritumoral hypodensity line and age. The nomogram and RF model yielded AUC of 0.951 (95% CI: 0.842–0.993) and 0.894 (95% CI: 0.766–0.966), respectively. The DeLong test found no statistically significant difference in diagnostic performance (p > 0.05), but DCA revealed that the nomogram surpassed the RF model in clinical usefulness. Conclusion: Two diagnostic prediction models based on a nomogram as well as RF method were reliable and easy-to-use for distinguishing between HP and GIST, which might also assist treatment planning.

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Incidence and Survival Outcomes of Gastrointestinal Stromal Tumors

Alvarez,

Piazuelo,

Fleitas-Kanonnikoff

et al. 2024

JAMA Netw Open

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ImportanceThe incidence of gastrointestinal stromal tumors (GISTs) increased after the implementation of GIST-specific histology coding in 2001, but updated data on trends and survival are lacking.ObjectiveTo examine the evolving epidemiology of GISTs in major organ sites.Design, Setting, and ParticipantsThis descriptive, population-based cohort study used nationally representative data from the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) Program, including the SEER-22 and SEER-17 registries. Data were from evaluated patients aged 20 years or older with GISTs diagnosed between January 1, 2000, and December 31, 2019. Analyses were last updated on October 29, 2023.Main Outcomes and MeasuresOrgan site-specific trends in age-standardized incidence rates and annual percent changes (APCs) in rates were estimated by race and ethnicity and, when possible, by sex, age, and primary indicator. Multivariable Cox proportional hazards regression models were used to examine racial and ethnic differences in overall and GIST-specific survival by site.ResultsThe SEER-22 and SEER-17 datasets contained 23 001 and 12 109 case patients with GISTs, respectively. Patients in the SEER-22 registry had a mean (SD) age of 64 (13) years and 51.3% were men. With regard to race and ethnicity, 9.7% of patients were Asian or Pacific Islander, 12.3% were Hispanic, 19.6% were non-Hispanic Black, and 57.7% were non-Hispanic White. Overall incidence rates of GISTs in the SEER-22 cohort increased substantially over time for all organ sites but the colon (APCs: esophagus, 7.3% [95% CI, 4.4% to 10.2%]; gastric, 5.1% [95% CI, 4.2% to 6.1%]; small intestine, 2.7% [95% CI, 1.8% to 3.7%]; colon, −0.2% [95% CI, −1.3% to 0.9%]; and rectum, 1.9% [95% CI, 0.1% to 3.8%]). There were similar increasing trends by age groups (&lt;50 vs ≥50 years), sex, race and ethnicity, and primary indicator for gastric and small intestine GISTs. Increases were mainly restricted to localized stage disease. Patients in the SEER-17 cohort had a mean (SD) age of 64 (14) years and 51.9% were men. With regard to race and ethnicity, 13.3% of patients were Asian or Pacific Islander, 11.6% were Hispanic, 17.8% were non-Hispanic Black, and 56.6% were non-Hispanic White. Non-Hispanic Black individuals had higher overall mortality for esophageal (adjusted hazard ratio [HR], 6.4 [95% CI, 2.0 to 20.3]) and gastric (adjusted HR, 1.4 [95% CI, 1.2 to 1.5]) GISTs compared with non-Hispanic White individuals. Asian or Pacific Islander individuals also had higher overall mortality for esophageal GISTs (adjusted HR, 5.6 [95% CI, 1.5 to 20.2]). Results were similar for GIST-specific survival.Conclusions and RelevanceIn this cohort study using SEER data, the incidence of GISTs in major organ sites increased in the last 2 decades among several population groups. These findings suggest that additional studies are warranted to identify risk factors, because histologic reclassification and higher availability of endoscopy and imaging do not fully explain these unfavorable incidence trends. Prevention efforts are needed to reduce the substantial survival disparities among racial and ethnic minoritized populations.

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NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022

Cited by 51 publications

References 76 publications

Jejunal Gastrointestinal Stromal Tumor: A Diagnostic Challenge

Jejunal Gastrointestinal Stromal Tumor: A Diagnostic Challenge

A CT-based nomogram established for differentiating heterotopic pancreas from gastrointestinal stromal tumor: compared with a machine-learning model

Incidence and Survival Outcomes of Gastrointestinal Stromal Tumors

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