Köksal BİLGEN, Sinan C. HASÇİÇEK, Süleyman ÇETİNKÜNAR, Bahadır CELEP, Serhat TOKGÖZ, Gündüz TUNÇ, Mesut Y. ATLI

Gastrointestinal stromal tümörlerde prognostik faktörler

Amaç: Bu çalışmada 2001-2006 yılları arasında S.B Ankara Dışkapı Yıldırım Beyazıt Eğitim ve Araştırma Hastanesi 1. Genel Cerrahi kliniğinde leiomyom, leiomyosarkom, leiomyoblastom, düz kas ya da nöronal farklılaşma gösteren stromal tümör ön tanılarıyla opere edilmiş ve GİST tanısı almış 25 olguda prognozu etkileyen faktörlerin araştırılması amaçlandı. Materyal ve Metot: Olguların genel ve hastalıksız sağ kalım süreleri hesaplandı. Genel hastalıksız, uzak hastalıksız ve lokal hastalıksız sağ kalımı etkileyen faktörler araştırıldı. Araştırılan parametreler: cins, yaş, yerleşim, kilo kaybı, anemi, palpabl kitle, ameliyat şekli, çap, mitoz, hücre tipi, nükleer atipi, nekroz, kanama, ülser, CD34, SMA, Kİ67, Fletcher prognostik index idi. Bulgular: Toplam 25 olgunun 15'i kadın (%60) 10'u (%40) erkektir. 25 olgunun klinik takipleri 1 ay ile 83 ay arasında değişmektedir. Histomorfolojik incelemede; mitoz oranı 50 BBA'da 1 ile 31 arasında değişmekte olup ortalama 3,2 mitozdur. CD117 25 olguda (%100), CD34 15 olguda (%60), SMA 8 olguda (%32), Desmin 1 olguda (%4), S100 1 olguda (%4) pozitif boyanmıştır. Ki-67'nin %10 un üzerinde pozitifliği 4 vakada izlenmiştir (%16). Fletcher prognostik indeksine göre; çok düşük risk gruplu 1 olgu (%4), düşük risk gruplu 8 olgu (%32), orta risk gruplu 5 vaka (%20), yüksek risk gruplu 11 vaka (%44) saptanmıştır. Sonuç: Gastrointestinal stromal tümörler için en önemli prognostik faktörün hastalığın tanı anındaki yaygınlığı olduğu kabul edilmektedir. Tek başına morfolojik parametreler bu tümörlerin klinik davranışını belirlemek için kullanılamaz. Çalışmamız sonucunda malign klinik seyir için yüksek riskin tayininde mide dışı yerleşim, 50 BBA'da 4 ve üzerinde mitoz sayısı, 10 cm ve üzerindeki çap majör prognostik parametreler olarak bulunmuştur. Ayrıca tanı anında kilo kaybı varlığı, anemi bulunması, Ki-67 pozitifliği de kötü prognozla ilişkilidir.

Anahtar Kelimeler:

Gastrointestinal neoplazmlar, Gastrointestinal stromal tümörler

Prognostic factors in gastrointestnal stromal tumors

Aim: This study aimed to determine factors affecting prognosis in 25 cases operated with prediagnosis of leiomyom, leiomyosarkom, leiomyoblastom, smooth muscle or stromal tumor showing neuronal differentiation and diagnosed with GIST at the Department of General Surgery, Dışkapı Training and Research Hospital, Ankara, Turkiye between the years 2001-2006. Material and Method: The general survival time and disease-free survival time of the cases were calculated. The factors affecting the survival were investigated. Sex, age, location, weight loss, anemia, palpabl mass, method of operation, diameter, mitosis, cell type, nuclear atypia, necrosis, haemorrhage, ulcer, CD34, SMA, KI67 and Fletcher prognostic index were the parameters evaluated. Results: Of the total 25 cases, 15 (60%) were female and 10 (40%) were male. The clinical follow-up of the 25 cases varied between 1 month and 83 months. In histomorphological examination, the rate of mitosis varied between 1 and 31 at 50 BBA with the average of 3.2 mitosis. CD117 was positively stained in 25 cases (100%), CD34 in 15 cases (60%), SMA in 8 cases (32%), Desmin in 1 case (4%) and S100 in 1 case (4%). The positive rate of Ki-67 over 10 % was observed in 4 cases (16%). It was determined according to Fletcher prognostic index that 1 patient was in the very low risk group (4%), 8 patients were in the low risk group (32%), 5 patients were in the medium risk group (20%) and 11 patients were in the high risk group (44%). Conclusion: It is accepted that the most important prognostic factor for gastrointestinal stromal tumors is the invasion degree of the disease at the time of diagnosis. The morphological parameters alone cannot be used to determine the clinical behaviour of these tumors. As a result of our study, location (except gastric location), 4 and more mitosis observed in BBA and a diameter of 10 cm and above were found to be the major prognostic parameters when determining the high risk of malignant clinical course. Besides, the existence of height loss, anemia and Ki-67 positivity at the time of diagnosis are associated with poor prognosis.

Keywords:

Gastrointestinal Neoplasms, Gastrointestinal Stromal Tumors,

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1. Fletcher CDM, Berman JJ, Corless C, Longley BJ, Remotti H, Rubin BP et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002;33(5):459-65.
2. Berman J, O'leary TJ. Gastrointestinal stromal tumor workshop. Hum Pathol 2001;32(6):578-82.
3. Mietinen M, Lasota J. Gastrointestinal stromal tumors definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438(1):1-12.
4. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: Recent advances in understanding of their biology. Hum Pathol 1999;30(10):1213-9.
5. Owen DA. The stomach. in: Stemberg S.S.(Ed). Diagnostic Surgical Pathology. Third edition. WW. Lippincott. Philadelphia. Volume II. Ch: 1999;32: 1337-9.
6. Cooper HS. Intestinal neoplasms. in: Sternberg S.S.(Ed). Diagnostic Surgical Pathology. Third edition. WW. Lippincott. Philadelphia. Volume II. Ch: 1999;34: 1454-7.
7. Ming SC, Goldman H. (Ed). Pathology of the Gastrointestinal Tract. Saunders, Philadelphia. Ch: 1992;15: 310-50.
8. Fenoglio-Preiser CM. (Ed). Gastrointestinal Pathology: an atlas and text. Second edition. Lippincott-Raven, Philadelphia. Ch: 1999;25: 1169-15.
9. Rosai T. Gastrointestinal tract., in: Rosai J. (Ed). Ackerman's Surgical Pathology. Eighth edition. Mosby, Philadelphia. Volume I. Ch: 11. S: 1996;645-7; 691-3.
10. Herrera GA, Cerezo L, Jones JA, Sack J, Grizzle WE, Pollack WJ et al. Gastrointestinal autonomic nerve tumors "plexosarcomas". Arch Pathol 1989;113(8):846-55.
11. Lauwers GY, Erlandson RA, Casper ES, Brennan MF, Woodruff JM. Gastrointestinal autonomic nerve tumors: A clinicopathologic, immunohistochemical and ultrastructural study of 12 cases. Am J Surg Pathol 1993;17(9):887-97.
12. Thomas JR, Mark RE, Libuit N. Gastrointestinal autonomic nerve tumor presenting as high-grade sarcoma. Digestive Diseases and Sciences 1994;39(9): 2051-2055.
13. Lee JR, Joshi V, Griffin JW, Lasota J, Miettinen M. Gastrointestinal autonomic nerve tumor. Am J Surg Pathol 2001;25(8):979-87.
14. Chang MS, Choe G, Kim WH, Kim Y. Small intestinal stromal tumors: A clinicopathologic study of 31 tumors. Pathol Int 1998;48(5):341- 7.
15. Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GlSTs) primary in the omentum and mesentery: Clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999;23(9):1109-18.
16. Yoneda E, Teramura K, Hiruma S, Satou T, Hashimoto S. Malignant gastrointestinal stromal tumor showing remarkable whorl formations. Arch Pathol Lab Med 2001;125(5):665-8.
17. Ishida T, Wada I, Horiuchi H, Oka T. ve Machinami R. Multiple small intestinal stromal tumors with skenoid fibers in association with neurofibromatosis 1 (von Recklinghausen's disease). Pathol Int 1996;46 (9):689-95.
18. M in KW. Small intestinal stromal tumors with skeinoid fibers: Clinicopathological, immunohistochemical, and ultrastructural investigations. Am J Surg Pathol 1992;16(2):145-55.
19. Evans HL. Smooth muscle tumors of gastrointestinal tract. A study of 56 cases followed for a minimum of 10 years. Cancer 1985;56 (9): 2242- 50.
20. Miettinen M, Blay JY, Sobin LH. Mesenchymal tumors of the stomach.In: Hamilton RS, Aaltonen LA (eds). WH0 classification tumors of the digestive system. Lyon: IARCPress 2000; 62-6.
21. Walsh NMG, Bodurtha A. Auerbach's myenteric plexus: a possible site of origin for gastrointestinal stromal tumors in von Recklinghausen's neurofibromatosis. Arch Pathol Lab Med 1990;114(5):522-5.