Gastrointestinal Stromal Tumors

Yıl 2009, Cilt: 16 Sayı: 4, 227 - 232, 01.08.2009

Oktay Büyükaşık A Oğuz Hasdemir Abuzer Dirican Cavit Çöl

Öz

Background: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumors of the gastrointestinal system. Origin, differentiation, classification, and prognosis of these tumors are controversial. Our aim in this study is to enroll our clinical experience about GIST by sharing the current literature information regarding the diagnosis and treatment of it. Patients and Method: Nine patients who was operated and pathologic examination were reported as GIST between 2003–2009 years have been viewed retrospectively. Patients' demographic, clinical, pathological features and treatment methods were evaluated. Results: There were 6 female and 3 male patients with mean age of 65.7±14.2 years. The origins of GIST were stomach in 4 cases (44%), small bowel in 3 (33%) and extra-intestinal in 2 (22%). Two patient's histological diagnosis were done preoperatively. The diagnoses of other patients were done postoperatively. Surgery was performed in emergency conditions in two cases due to complications of tumor. An unresectable patient underwent curative surgery after development on partial remission with imatinib treatment. Four (44%) cases were identified as high risk group, 4 (44%) cases as intermediate, and 1 (11%) case as low risk group according to biological behavior of tumors. Conclusion: Although essential treatment of GIST is surgery, long disease-free survival time and important survival achievements were obtained with the use of tyrosine kinase inhibitors. Ensuring early diagnosis of these tumors and ability of diagnosis with advanced immunohistochemical methods are obstacles to be overcome. Key words: Gastrointestinal Stromal Tumors, Imatinib, Surgical Treatment

Anahtar Kelimeler

Gastrointestinal Stromal Tumors, Imatinib, Surgical Treatment

Gastrointestinal Stromal Tümörler

Öz

Amaç: Gastrointestinal stromal tümör (GİST), gastroinestinal sistemin en sık görülen mezenşimal tümörüdür. Bu tümörlerin kökeni, diferansiyasyonu, sınıflaması ve prognozu tartışmalıdır. Bu çalışmada GİST'le ilgili klinik deneyimlerimizi aktararak, tanı ve tedavisi ile ilgili güncel literatür bilgilerinin paylaşılması amaçlandı. Gereç ve Yöntem: 2003-2009 tarihleri arasında cerrahi girişimde bulunulan ve patolojik tanısı GİST olan 9 olgu retrospektif olarak incelendi. Hastaların demografik, klinik, patolojik özellikleri ve tedavi yöntemleri değerlendirildi. Bulgular: Hastaların 6'sı kadın, 3'ü erkek olup yaş ortalaması 65.7±14.2'idi. Olguların 4'ü (%44) mide, 3'ü (%33) ince barsak ve 2'si (%22) ekstraintestinal kaynaklı idi. İki hastaya preoperatif histolojik tanı kondu. Diğer hastaların tanısı postoperatif konuldu. İki olguya tümörün komplikasyonları nedeniyle acil koşullarda cerrahi girişim uygulandı. Unrezektabl bir olguya imatinib tedavisi sonrası parsiyel remisyon gelişmesi üzerine küratif cerrahi girişim uygulandı. Olgular tümörün biyolojik davranışı yönünden değerlendirildiğinde, 4 hastanın (%44) yüksek risk grubunda, 4 hastanın (%44) orta risk grubunda, bir olgunun ise (%11) düşük risk grubunda olduğu saptandı. Sonuç: GIST'in esas tedavi yöntemi cerrahi olmakla birlikte, tirozin kinaz inhibitörlerinin kullanımı ile hastalıkla iyi yaşam süresi, hastalıksız yaşam süresi ve sağ kalımda önemli başarılar elde edilmiştir. Bu tümörlerin erken tanısının sağlanması ve ileri immünhistokimyasal yöntemlerle tanısının konabilmesi, aşılması gereken engeller olarak durmaktadır. Anahtar kelimeler: Gastrointestinal Stromal Tümör, İmatinib, Cerrahi Tedavi

Anahtar Kelimeler

Gastrointestinal Stromal Tümör, İmatinib, Cerrahi Tedavi

Kaynakça

• Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 2002;33:459-65.
• Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis.Am J Surg Pathol 1983;7:507-19.
• Miettinen M, Makhlouf H, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up. Am J Surg Pathol 2006;30:477-89.
• Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol 2000;7:705-12.
• Doğanavsargil B, Akalın T, Sezak M, Kandiloğlu G, Tunçyürek M.Gastrointestinal stromal tümörlerde histopatolojik değerlendirme “Ege Üniversitesi Tıp Fakültesi Patoloji Anabilim Dalı’nın 1992-2007 yılları arasındaki deneyimi” Ege Tıp Dergisi 2008; 47 :163-170.
• Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998;152:1259-69.
• Gupta P, Tewari M, Shukla HS. Gastrointestinal stromal tumor. Surg Oncol 2008;17:129-38.
• Nilsson B, Bümming P, Meis-Kindblom JM, Odén A, Dortok A, Gustavsson B, Sablinska K, Kindblom LG. Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era--a population- based study in western Sweden. Cancer 2005;15;103:821-29.
• DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000;231:51-8
• Miettinen M, Lasota J. Gastrointestinal stromal tumors-- definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-12.
• Biasco G, Velo D, Angriman I, Astorino M, Baldan A, Baseggio M, Basso U, Battaglia G, et all. Gastrointestinal stromal tumors: report of an audit and review of the literature. Eur J Cancer Prev 2009;18:106-16.
• Nishida T, Hirota S. Biological and clinical review of stromal tumors in the gastrointestinal tract. Histol Histopathol 2000;15:1293-301.
• Krska Z, Pesková M, Povýsil C, Horejs J, Sedlácková E, Kudrnová Z. GIST of pancreas. Prague Med Rep 2005;106(2):201-8 (Abstract).
• Yan BM, Pai RK, Van Dam J. Diagnosis of pancreatic gastrointestinal stromal tumor by EUS guided FNA. JOP 2008;9:192-96.
• Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, Sobin LH. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999;23:1109-118
• Dizdar Ö, Güler N. Gastrointestinal stromal tümörler ve imatinib (Glivec®) tedavisi Hacettepe Tıp Dergisi 2004; 35:87-91.
• Miettinen M, El-Rifai W, Sobin LH, Lasota J. Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a rewiew. Human Pathology 2002; 33: 478-483.
• Ernst S, Hubbs A, Przygodzki R, Emory T, Sobin L, O’Leary T. KIT mutation portends poor prognosis in gastrointestinal stromal/smooth muscle tumors. Lab Investi 1998;78:1633–636.
• Taniguchi M, Nishida T, Hirota S, Isozaki K, Ito T, Nomura T, Matsuda H, Kitamura Y. Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors. Cancer Res 1999;59:4297-300.
• Gold JS, van der Zwan SM, Gönen M, Maki RG, Singer S, Brennan MF, Antonescu CR, De Matteo RP. Outcome of metastatic GIST in the era before tyrosine kinase inhibitors. Ann Surg Oncol 2007;14:134-42. Erratum in: Ann Surg Oncol 2007;14:3027.
• Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg 2006;244(2):176-84.
• Dimetri GD, Joensuu H. Systemic treatment of patients with gastrointestinal stromal tumor: Current status and future opportunities. EJS supp. 2008;6:1–14.
• Din OS, Woll PJ. Treatment of gastrointestinal stromal tumor: focus on imatinib mesylate. Ther Clin Risk Manag 2008;4:149-62.