Incidence of gastrointestinal stromal tumours is underestimated: Results of a nation-wide study

Goettsch, Wim; Bos, Steffan D.; Breekveldt-Postma, Nancy S; Casparie, Mariël K.; Herings, Ron M. C.; Hogendoorn, Pancras C.W.

doi:10.1016/j.ejca.2005.09.009

Cited by 266 publications

(213 citation statements)

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“…[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.…”

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confidence: 99%

Gastrointestinal stromal tumors: what do we know now?

2014

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Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the GI tract, arising from the interstitial cells of Cajal, primarily in the stomach and small intestine. They manifest a wide range of morphologies, from spindle cell to epithelioid, but are immunopositive for KIT (CD117) and/or DOG1 in essentially all cases. Although most tumors are localized at presentation, up to half will recur in the abdomen or spread to the liver. The growth of most GISTs is driven by oncogenic mutations in either of two receptor tyrosine kinases: KIT (75% of cases) or PDGFRA (10%). Treatment with tyrosine kinase inhibitors (TKIs) such as imatinib, sunitinib, and regorafenib is effective in controlling unresectable disease; however, drug resistance caused by secondary KIT or PDGFRA mutations eventually develops in 90% of cases. Adjuvant therapy with imatinib is commonly used to reduce the likelihood of disease recurrence after primary surgery, and for this reason assessing the prognosis of newly resected tumors is one of the most important roles for pathologists. Approximately 15% of GISTs are negative for mutations in KIT and PDGFRA. Recent studies of these so-called wild-type GISTs have uncovered a number of other oncogenic drivers, including mutations in neurofibromatosis type I, RAS genes, BRAF, and subunits of the succinate dehydrogenase complex. Routine genotyping is strongly recommended for optimal management of GISTs, as the type and dose of TKI used for treatment is dependent on the mutation identified.

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confidence: 99%

Gastrointestinal stromal tumors: what do we know now?

2014

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“…Most cases are the result of sporadic mutations, however familial GISTs have been described, and appear to be associated with earlier development and increased number of GISTs (Miettinen et al, 2001;Goettsch et al, 2005;Janeway et al, 2007;. We recorded one patient with this familial form, presenting with a GIST localized to the stomach at age 60 with two recurrences, both successfully managed with resection.…”

Section: Discussionmentioning

confidence: 97%

Gastrointestinal Stromal Tumours: A 10 Year Multicenter Audit

Watson¹,

Kelly²,

Malone³

et al. 2016

CCO

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Background: Gastrointestinal stromal tumours (GISTs) are unique neoplasms of the gastrointestinal (GI) tract. The development of targeted therapeutic agents such as imatinib mesylate (Glivec) has altered the way on how we now manage these rare malignancies. The aim of this study was to evaluate the management of GISTs in three Irish tertiary hospitals. Methods: We performed a retrospective, multicenter audit of patients diagnosed with gastrointestinal stromal tumours over a ten year period (2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015). Results: 110 patients were included in the study. Abdominal pain was the most common presenting symptom, reported in 30% of patients, while 31% were incidental findings. The stomach was the most common primary site of disease, observed in 77% of cases. 15 patients had metastatic disease at the time of diagnosis (14%), and 10 of these patients had liver involvement. More than half of patients (61%) were managed with surgical excision alone (61%), while 24 were managed with surveillance and 28 patients treated with adjuvant Glivec, which was generally well tolerated. 18 patients (20%) demonstrated recurrent or progressive disease after first line treatment. 102 patients (93%) are alive today. Conclusion: While surgery is widely regarded as the primary treatment modality for GISTs the addition of imatinib mesylate has enabled physicians to deliver more personalised treatment while optimising patient outcomes.

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“…However, there seems close overlap been the low and very low risk groups. This may be related to the small number of patients in this study but this close overlap has been noted previously in relation to recurrence free survival in a study comparing the NIH and AFIP criteria [20]. In a modified AFIP system the low risk groups were combined and a new very high risk category was created which consisted of gastric GISTs >10 cm and mitotic rate >5/50 HPF.…”

Section: Discussionmentioning

confidence: 99%