Zeitschrift für Gastroenterologie
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Practice Guideline Guideline
[S3-Guidelines Conference "Colorectal Carcinoma" 2004].
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Gastrointestinal neuroendocrine tumours are classified as functioning or non-functioning according to the presence or absence of a clinically evident hypersecretion syndrome. In foregut tumours the presence of autonomous hormone secretion and the respective hypersecretion syndrome indicate functionality. Abdominal ultrasound (US), computed tomography (CT), magnetic resonance tomography (MRT) and somatostatin receptor scintigraphy (SRS) are used for localisation of the primary tumour and metastasis. ⋯ Chemotherapy is less effective in midgut tumours compared to foregut tumours. Cytoreductive strategies (chemoembolisation, thermo- or cryotherapy, cytoreductive surgery) and radio-receptor therapy may offer new therapeutic options. However, their definitive value has yet to be defined.
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The vast majority of mesenchymal tumors originating from the GI tract consists of gastrointestinal stromal tumors (GIST), an entity just recently defined. The incidence is estimated to be around 10 - 20/1000000, the median age at diagnosis has been reported to be 55 to 65 years. GISTs most commonly occur in the stomach or duodenum, followed by the small intestine. ⋯ Early results from clinical trials with response rates around 60 % and progression arrest in more than 80 % of patients resulting in fast relief of symptoms, confirm the high activity of this novel treatment. The role of adjuvant treatment after potentially curative resection of GIST is currently evaluated in ongoing clinical trials. Patients with progressive disease while under treatment with Imatinib should be enrolled in studies testing novel treatment strategies as RAD001, PKC412 or SU11 248.
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Three cases of gastrointestinal stromal tumors (GIST) are reported as typical examples of the broad clinical spectrum in which these rare tumors can be detected. The first case describes an 82-year-old patient with a hemorrhagic shock due to upper gastrointestinal bleeding from a GIST of the stomach. GIST most frequently present with either gastrointestinal bleeding, abdominal pain or a detectable mass on physical examination or by ultrasound imaging. ⋯ The cases are used to discuss the consequences for therapy and prognosis resulting from the heterogeneity of this tumor entity; the relevant immunohistochemical markers used to distinguish between various tumor subtypes of gastrointestinal mesenchymal tumors (GIMT) are listed. Since gastrointestinal stromal tumors (GIST) represent the most common subgroup of GIMT, we focus on the clinicopathological prognostic factors of GIST. The third case of a 40-year-old patient with a malignant GIST recurrence after surgery and exhibiting secondary resistance after one year of successful therapy with the receptor tyrosine kinase inhibitor imatinib (Gleevec), antagonizing pathogenetically relevant constitutive c-KIT activation, illustrates the potential and limitations of the only effective drug treatment for advanced GIST.