Review article: the biology, diagnosis and management of gastrointestinal stromal tumours

Abstract

SUMMARY BackgroundGastrointestinal stromal tumours (GIST) are the most common mesenchymal tumours of the gastrointestinal tract with an increasing incidence. AimsTo review the biology, diagnosis and treatment of gastrointestinal stromal tumours. MethodsA PubMed search using the phrases 'Gastrointestinal stromal tumor', 'imatinib', 'c-kit'. ResultsThe diagnosis of GIST is established by histology supplemented by the immunohistochemical marker CD117, which is positive in 95% of cases. The most common site of the tumour is the stomach. Most GIST are benign with 20-30% malignant. Five-year survival for malignant GIST ranges between 35% and 65% and depends primarily on tumour size, mitotic index and location. The malignant behaviour of GIST is best assessed by invasion of adjacent structures and distant metastases. The gold standard for treatment is surgical resection. Imatinib, a tyrosine kinase inhibitor, is the primary therapy for unresectable, recurrent or metastatic disease. ConclusionsGastrointestinal stromal tumours are rare tumours of the gastrointestinal tract and they vary in presentation. When surgical resection is not achievable, imatinib is the treatment of choice.

Partial response to imatinib treatment in a patient with unresectable gastrointestinal stromal tumor: A case report and mini literature review

“…Exons 13 and 17 rarely exhibit these mutations, while exons 18 and 12 show lower mutation rates for PDGFRα of 5 and 1%, respectively (5). The clinical manifestations of GISTs are highly variable and depend on tumour location, size and growth pattern (7). The most common presenting symptoms are associated with gastrointestinal bleeding, including melena, hematemesis or symptomatic anaemia, while other symptoms may include abdominal fullness, early satiety and a palpable mass or pain (7).…”

“…The clinical manifestations of GISTs are highly variable and depend on tumour location, size and growth pattern (7). The most common presenting symptoms are associated with gastrointestinal bleeding, including melena, hematemesis or symptomatic anaemia, while other symptoms may include abdominal fullness, early satiety and a palpable mass or pain (7). Among patients with GIST, 15-50% present with overt metastasis, and the most common sites of metastases are the peritoneum and liver (8).…”

The functional morphology and role of cardiac telocytes in myocardium regeneration

“…The c-kit (CD117 antigen), a protein transmembrane protein kinase receptor, is essential for telocyte function and represents the first routinely used marker for its identification. Originally, it was used for identification of ICCs in the gut and for tumors derived from these cells; approximately 95% of gastrointestinal stromal tumors cases are positive for CD117 antigen (Miettinen et al 2002;Iorio et al 2014). …”

Contribuição dos aspectos endoscópicos e ecoendoscópicos para o diagnóstico diferencial das lesões subepiteliais gástricas

“…[26][27][28][29] Portanto, frente à LSE gástrica, deve-se considerar a possibilidade diagnóstica de GIST, dada sua predileção pelo sítio gástrico e pelo impacto à conduta terapêutica que esse diagnóstico provoca, pois são atualmente consideradas lesões malignas ou potencialmente malignas. 30 Dessa forma, os GIST designam um grupo de tumores mesenquimais GI que originam-se da parede do TGI, mesentério, omento ou retroperitônio, e englobam a maioria dos tumores previamente classificados como neoplasias gastrointestinais de músculo liso, devido à característica histológica de células fusiformes e aparente origem na camada muscular própria da parede do trato digestivo. 1 À histologia, a maioria dos GIST são do tipo de células fusiforme (70%), indistinguível da apresentação histológica de um leiomioma, e apenas o painel imuno-histoquímico pode efetuar a diferenciação diagnóstica.…”

“…Em 20% dos casos apresentam-se como tipo histológico epitelióide, ou misto em 10%. 30 Em 1998, a observação de que os GIST expressam CD117 (receptor de membrana proteína tirosina-quinase KIT, ou simplesmente proteína c-kit), assim como CD34 (glicoproteína transmembrana e antígeno de célula progenitora hematopoiética encontrada em células mesenquimais), compartilhando marcadores similares com as células intersticiais de Cajal, foi passo fundamental para o início da elucidação da gênese e classificação correta dos tumores mesenquimais. 31 …”