Gastrointestinal stromal tumors: The incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era

Abstract

BACKGROUND Recent breakthroughs regarding gastrointestinal stromal tumors (GIST) and their pathogenesis have redefined diagnostic criteria and have led to the development of molecularly targeted drug therapy. New treatment options mandate more accurate information regarding the incidence, prevalence, clinical behavior, and prognostic factors of GIST. METHODS All patients (n = 1460) who potentially had GIST diagnosed from 1983 to 2000 in western Sweden (population, 1.3–1.6 million) were reviewed, and 288 patients with primary GIST were identified. The incidence and prevalence of GIST were determined, and predictive prognostic factors, including current risk‐group stratifications, were analyzed statistically. RESULTS Ninety percent of GISTs were detected clinically due to symptoms (69%) or were incidental findings at surgery (21%); the remaining 10% of GISTs were found at autopsy. Forty‐four percent of symptomatic, clinically detected GISTs were categorized as high risk (29%) or overtly malignant (15%), with tumor‐related deaths occurring in 63% of patients and 83% of patients, respectively (estimated median survival, of 40 months and 16 months, respectively). Tumor‐related deaths occurred in only 2 of 170 of patients (1.2%) with very‐low‐risk, low‐risk, or intermediate‐risk tumors. The annual incidence of GIST was 14.5 per million. The prevalence of all GIST risk groups was 129 per million (31 per million for the high‐risk group and the overtly malignant group). CONCLUSIONS GIST has been under recognized: Its incidence, prevalence, and clinical aggressiveness also have been underestimated. Currently existing risk‐group stratification systems based on tumor size and mitotic rate delineate GIST patients who have a poor prognosis. Prognostication in patients with GIST can be refined using a proposed risk score based solely on tumor size and proliferative index. Cancer 2005. © 2005 American Cancer Society.

Mutational spectrum and therapy response of metastasized GIST in Central Switzerland – A population-based study

“…Gastrointestinal stromal tumours (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract with an incidence of approximately 0.65 -1.5 cases per 100'000 inhibitants (1)(2)(3)(4)(5)(6). The most frequently affected sites are stomach and small intestine, but GISTs may also develop in any other part of the GI tract, peritoneum and retroperitoneum.…”

Gastrointestinal stromal tumors: what do we know now?

“…[1][2][3][4] This corresponds to between 3300 and 6000 new cases per year in the United States. Following surgical resection, GISTs often recur locally, spread diffusely throughout the serosal surfaces of the abdomen, and/or metastasize to the liver.…”

Tissue microarrays characterise the clinical significance of a VEGF-A protein expression signature in gastrointestinal stromal tumours

“…The uncertain prognosis of GIST, both before (Nilsson et al, 2005) and after (Kosmadakis et al, 2005) imatinib treatment, indicate the need for the search of other molecular prognostication biomarkers. GIST are highly vascularised neoplasms and VEGF-A is a major antiangiogenic therapeutic target (Ferrara and Kerbel, 2005).…”