Gastrointestinal Stromal Tümör
Özet
Gastrointestinal stromal tümörler (GIST'ler) gastrointestinal sistemin nadir görülen mezenkimal neoplazmlarıdır. Gastrointestinal sistemin pacemaker hücreleri olarak da adlandırılan Cajal hücrelerinden kaynaklanırlar. Çoğu GIST, KIΤ veya РDGFRA mutasyonu taşırken süksinat dehidrojenaz (SDH) veya diğer bazı genlerdeki mutasyonlar da daha az sıklıkta görülebilmektedir (1,2). GIST'ler genellikle yaşlı erişkinlerde görülür ve ortalama tanı yaşı 65 ile 69 arasındadır (3,4).Kadın ve erkek hastalarda benzer oranda görülmektedir (3).
GIЅΤ şüphesi olan hastalarda, primer tümörün ilk değerlendirmesi için bilgisayarlı tomografi (BΤ) kullanılması önerilir. Manyetik rezonans görüntüleme (MRI), BT kontrastı alamayan veya rektal primeri olan hastalarda bir alternatif olabilir. GIST'ler değişken biyolojik hastalık davranışı sergileyebilmesine rağmen hepsinin metastatik hastalığa dönüşme potansiyeli vardır. Mevcut klinik risk faktörlerine dayanarak, rekürrens ve metastazın göreceli risklerini tahmin etmek için prognostik modeller geliştirilmiştir (50,62,63). Gastrointestinal stromal tümörlerde tedavinin temelini cerrahi oluştururken adjuvan ve metastatik ilk hatta sistemik tedavide imatinib kullanılmaktadır.
Gastrointestinal stromal tümörlerin tedavisi sonrası takibin önemi oldukça büyük olup takibin nasıl yapılması gerektiği konusunda net bir konsensüs olmamakla birlikte çok düşük riskli hastalık grubu dışında takibin esas parametrelerini kontrastlı batın-pelvis BT veya kontastlı/kontrastsız MR oluşturur.
Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasias of the gastrointestinal tract. They differ from Cajal, which is also presented as a gastrointestinal system pacemaker system. While most GISTs carry KIΤ or РDGFRA mutations, mutations in succinate dehydrogenase (SDH) or some other genes can also be seen less frequently (1-2). GIST’s usually occur in older adults, with the average age at diagnosis between 65 and 69 years. It is seen at similar rates in male and female patients (3,4). In patients with suspected GIЅΤ, the use of computed tomography (CΤ) is recommended for initial evaluation of the primary tumor. Magnetic resonance imaging (MRI) may be an alternative in patients who cannot receive CT contrast or who have a rectal primary.
Although GISTs can exhibit variable biological disease behavior, all have the potential to develop into metastatic disease. Based on existing clinical risk factors, prognostic models have been developed to estimate the relative risks of recurrence and metastasis (50,62,63). While surgery forms the basis of treatment in gastrointestinal stromal tumors, imatinib is used in adjuvant and metastatic first line and even systemic treatment.
The importance of follow-up after the treatment of gastrointestinal stromal tumors is great, and although there is no clear consensus on how follow-up should be done, the main parameters of follow-up, except for very low-risk disease groups, are contrast-enhanced abdomen-pelvis CT or MRI with/without contrast.
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