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1、胃肠道间质瘤概念,胃肠道间质瘤（Gastrointestinal Stromal Tumors, GIST）是一类起源于胃肠道间叶组织的肿瘤，占消化道间叶肿瘤的大部分。Mazur 等于1983 年首次提出了胃肠道间质肿瘤这个概念。,发病率及发病部位,Gastrointestinal stromal tumours (GISTs) represent 0.1-3% of gastrointestinal malignancies and 80% of gastrointestinal mesenchymal tumours The prevalence of the disease is est
2、imated at 11 to 20 cases per million of population 1、2、3.,Sixty percent of GISTs present in the stomach, 30% in the small intestine, 5% in the colon and rectum and 5% in the oesophagus About 140 new cases are recognized in Greece every year; the mean age of diagnosis is 60 years and the male to fema
3、le ratio approximately 1.4:1 4.,GIST的症状、体征,The delayed diagnosis of stromal tumours, due to the lack of specific symptoms, results in 25% of patients presenting with metastatic disease. It often takes up to 6 months from the onset of initial symptoms to reach a diagnosis 5.,The most frequent manifes
4、tations of the disease include gastrointestinal haemorrhage, fatigue due to chronic anaemia, loss of appetite, weight loss, vom-iting, the presence of a palpable mass, and non-spe-cific abdominal pain. These symptoms appear due to displacement, rather than infiltration of nearby structures. A combin
5、ation of symptoms is present in over 5% of patients 6、7,病理诊断基因诊断,The kit protein is expressed in 90-95% of GISTs. This protein represents a transmembrane receptor that acts like a tyrosine kinase. In 5-10% of GISTs, mutations in Platelet-Derived Growth Factor Recep-tor (PDGFR and PDHFR) are present
6、that are mutually exclusive to the KIT mutations, while some GISTs have no detectable mutations in either receptor (wild-type GISTs) 8,9.,The expression ofCD34 is more frequent in gastric GISTs (85%), while it is positive in only 50% of GISTs that arise from the small intestine 10. CD34 is universal
7、ly expressed in oesophageal GISTs. Another recently recognized marker is cyclooxygenase 2 (COX-2) At least 80% of GISTs express this protein 11,病理图示,The majority of stromal tumours (70%) consist of spindle cells, 20% are made up of epithelioid cells and the rest present with mixed morphology,CT and
8、EUS,Computed tomography (CT) and endoscopic ultrasound (EUS) have contributed significantly to the diagnosis of GISTs. Computed tomography repre-sents the best imaging modality for tumours 5 cm, while EUS is most useful in identifying tumours 10 cm in size with 5 mitoses per high-powered field (HPF)
9、 tend to recur, metastasize more frequently and are related with poor results. These characteristics are observed in 20-30% of patients with GISTs. Other prognostic factors include the location of the tumour, the R0 excision of the tumour and the mutations of the c-kit gene 19.,Other prognostic fact
10、ors include the location of the tumour, the R0 excision of the tumour and the mutations of the c-kit gene 20.,文章内容出处,1. Burkill GJ, Badran M, Al-Muderis O, Meirion Thomas J, Jud- son IR, Fischer C, Moskovic EC. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of
11、metastatic spread. Radiology 2003; 226: 527-532 2. Duffaud F, Blay JY. Gastrointestinal stromal tumors: biology and treatment. Oncology 2003; 65: 187-197 3. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors. Re- currence patterns and progn
12、ostic factors for survival. Ann Surg 2000; 231: 51-58 4. Goettsch WG, Bos SD, Breekveldt Postma N, Casparie M, Her- ings RM, Hogendoorn PC. Incidence of gastrointestinal stromal tumors is underestimated: results of a nation-wide study. Eur J Cancer 2005; 41: 2868-2872 5. Kosmadakis N, Visvardis EE, Kartsaklis P, Tsimara M, et al. The role of surgery in the management of gastrointestinal stromal tumors (GISTs) in the era of imatinib mesylate effectiveness. Surg Oncol 2005; 14: 75-84,
