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Tumores do estroma gastrointestinal (GIST) : trabalho de revisão
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Os tumores do estroma gastrointestinal (GIST) são neoplasias raras com origem nas células intersticiais de Cajal. Embora relativamente raro, os GISTs representam 80% dos tumores mesenquimais do sistema digestivo.
Os GISTs são frequentemente assintomáticos, sendo diagnosticados incidentalmente por exames de imagem ou endoscópicos.
Estes tumores constituem uma entidade distinta com base na sua patogénese molecular, coloração imuno-histoquímica e capacidade de resposta à terapêutica alvo.
A ressecção cirúrgica completa, sem linfadenectomia, é considerada o tratamento padrão dos GISTs primários localizados (sem evidência de disseminação peritoneal ou metastização à distância). A inibição KIT/PDGFRΑ com inibidores tirosina-quinases (Imatinib e Sunitinib) permite o controlo de GISTs inoperáveis e/ou metastáticos. A taxa de resposta clínica ao Imatinib e Sunitinib parece depender da presença e do tipo de mutações nos genes KIT e PDGFRΑ.
Este artigo inclui uma revisão da epidemiologia, características clínicas, marcadores imuno-histoquímica, diagnóstico diferencial, prognóstico e opções de tratamento actualmente empregues neste tipo de tumor.
The gastrointestinal stromal tumors (GIST) are rare neoplasms originated in the interstitial cells of Cajal. Although relatively rare, GISTs represent 80% of the mesenchymal tumors of the digestive system. GISTs are often asymptomatic, being diagnosed incidentally by imaging or endoscopy. These tumors represent a distinct entity based on their molecular pathogenesis, Immunohistochemical staining and responsiveness to target therapy. Complete surgical resection, without lymphadenectomy, is considered the standard of treatment to localized primary GISTs (there is no evidence of peritoneal dissemination or metastatic disease). KIT /PDGFRα inhibition with Imatinib and Sunitinib inhibitors allows the control of inoperable and/or metastatic GISTs. The rate of clinical response to Imatinib and Sunitinib appears to depend on the presence and type of mutacions in the KIT and PDGFR genes. This article includes a review of the epidemiology, clinical characteristics, immunohistochemical markers, differential diagnosis, prognosis and treatment options which are currently used in this type of tumors.
The gastrointestinal stromal tumors (GIST) are rare neoplasms originated in the interstitial cells of Cajal. Although relatively rare, GISTs represent 80% of the mesenchymal tumors of the digestive system. GISTs are often asymptomatic, being diagnosed incidentally by imaging or endoscopy. These tumors represent a distinct entity based on their molecular pathogenesis, Immunohistochemical staining and responsiveness to target therapy. Complete surgical resection, without lymphadenectomy, is considered the standard of treatment to localized primary GISTs (there is no evidence of peritoneal dissemination or metastatic disease). KIT /PDGFRα inhibition with Imatinib and Sunitinib inhibitors allows the control of inoperable and/or metastatic GISTs. The rate of clinical response to Imatinib and Sunitinib appears to depend on the presence and type of mutacions in the KIT and PDGFR genes. This article includes a review of the epidemiology, clinical characteristics, immunohistochemical markers, differential diagnosis, prognosis and treatment options which are currently used in this type of tumors.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2015
Palavras-chave
Tumores do estroma gastrointestinal Células intersticiais de Cajal Gastroenterologia