Gastrointestinální stromální tumor – analýza vlastní sestavy pacientů, přehled literatury

Authors: R. Pohnán;  M. Ryska;  R. Doležel;  I. Veverová 1;  Z. Linke 2
Authors‘ workplace: Chirurgická klinika 2. LF UK a ÚVN Praha, přednosta prof. MUDr. Miroslav Ryska, CSc. ;  Oddělení patologie ÚVN Praha, primář MUDr. Petr Hrabal 1;  Radioterapeuticko-onkologické oddělení FN Motol Praha, primářka MUDr. Jana Prausová 2
Published in: Rozhl. Chir., 2009, roč. 88, č. 11, s. 629-633.
Category: Monothematic special - Original


Gastrointestinal stromal tumors (GIST) are common mesenchymal gastrointestinal tumors, however, their incidence rate is low. The tumors originate from progenitor cells of interstitial cells of Cajal-gastrointestinal pacemaker cells, and the majority of them express c-Kit, a tyrosin kinase receptor. The aim of this study was to assess the GIST treatment in a group of patients and to compare the outcomes with literature data.

The authors performed a retrospecitve analysis of all patients with histologically confirmed GISTs, who were operated in the 2nd Surgical Clinic of the Charles University Medical Faculty (LF UK) in Prague and in the Central Military Hospital Prague (ÚVN Praha), from 2003 to 2008.

During the five-year period, 13 patients underwent surgery in the Central Military Hospital Prague. The commonest tumor locations were the following: stomach (46%), small intestine (duodenum 23%, jejunum 23%, ileum 8%). R0 resection was performed in 12 subjects (92%). 10 patients (77%) remain in remission, in one patient, the disease is stabilized (8%), and in one patient, the disease progression and generalization has been recorded (8%).

Surgery is a standard treatment in localized tumors. Following radical resection, the patients benefit from adjacent treatment with tyrosin kinase inhibitors. Specific tyrosin kinase inhibitors have been shown effective in the treatment of metastatic and relapsing disorders. Primary surgical treatment in metastatic diseases remains a paliative option for patients with bleeding and obstruction.

Key words:
gastrointestinal stromal tumor – diagnostics – surgery


1. Nilsson, B., Bumming, P., Meis-Kindblom, J. M., Odén, A., Dortík, A., Gustavsson, B., Sablinska, K., Kindblom, L. G. Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era – a population based study in western Sweden. Cancer, 2005; 103: 821–829.

2. Kindblom, L. G., Meis-Kindblom, J. M., Bümming, P. Incidence, prevalence, phenotype and biologic spektrum of gastrointestinal stromal cell tumors (GIST): a population-based study of 600 cases. Ann. Oncol., 2002; 13: 157.

3. Mazur, M. T., Clark, H. B. Gastric stromal tumours. Reappraisal of histogenesis. Am. J. Surg. Pathol., 1983; 7: 507–519.

4. Kindblom, L. G., Remotti, H. E., Aldenborg, F., Meis-Kindblom, J. M. Gastrointestinal Pagemaker cell tumor (GIPACT): Gastrointestinal stromal tumors show phenotypic characteristics of the intestinal cells of Cajal. Am. J. Pathol., 1998; 30: 1213–1220.

5. van der Zwan, S. M., Dematteo, R. P. Gastrointestinal stromal tumor: 5 years later. Cancer, 2005; 104: 1781–1788.

6. Miettinen, M., Monihan, J. M., Sarlomo-Rikala, M., et at. 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: 1109–1118.

7. Mendosa-Marin, M., Hoang, M. P., Albores-Saavedra, J. Malignant stromal tumor of the gallblader with intestinal cell sof Cajal phenotype. Arch. Pathol. Lab. Med., 2002; 126: 481–483.

8. Daum, O., Klecka, J., Ferda, J., et al. Gastrointestinal stromal tumor of the pankreas: case report with documentation of KIT gene station. Wirchows arch., 2005; 446: 470–472.

9. Lasota, J., Carlsson, J. A., Miettinen, M. Spindle cell tumor of urinary bladder serosa with phenotypic and genotypic features of gastrointestinal stromal tumor. A clinical report with documentation of KIT expression and mutation. Arch. Pathol. Lab. Med., 2000; 124: 894–897.

10. Ceballos, K. M., Francis, J. A., Mazurka, J. L. Gastrointestinal stromal tumor presenting as a recurrent vaginal mass. Arch. Pathol. Lab., 2004; 128: 1442–1444.

11. Din, O. S., Woll, P. J. Treatment of gastrointestinal stromal tumor: focus on imatinib mesylate. Therapeutics and clinical risk man. 2008; 4: 149–162.

12. Bucher, P., Villiger, P., Egger, J. F., Buhler, L. H., Morel, P. Management of gastrointestinal stromal tumours: from diagnosis to treatment. Swiss Med. Wkly., 2004; 134: 145–153.

13. Daum, O., Vaněček, T., Šíma, R., Michal, M. Gastrointestinal stromal tumor: update. Klinická Onkologie, 2006; 19: 203–211.

14. Corless, C. L., Fletcher, J. A., Heinrich, M. C. Biology of gastrointestinal stromal tumors. J. Clin. Oncol., 2004; 22: 3813–3825.

15. Sarlomo-Ricala, M., Kovatich, A. J., Barusevicius, A., Miettinen, M. CD 117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD 34. Mod. Pathol., 1998; 11: 728–734.

16. Miettinen, M., Majidi, M., Lasota, J. Pathology and diagnostic kriteria of gastrointestinal stromal tumors (GISTs): a review. Eur. J. Cancer, 2002; 38: S39–S51.

17. Fletcher, C. D. M., Berman, J. J., Corles, C., at al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum. Pathol., 2002; 33: 459–465.

18. Emory, T. S., Sobin, L. H., Lukes, L., et al. Prognosis of gastrointestinal smoot-muscle (stromal) tumors: dependance on anatomic site. Am. J. Surg. Pathol., 1999; 23: 82–87.

19. Miettinen, M., Lasota, J. Gastrointestinal stromal tumors – definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Wirchows arch., 2001; 438: 1–12.

20. De Matteo, R. P., Lewis, J. J., Leung, D., Mudan, S. S., Woodruff, J. M., Brennan, M. F. Two hundred gastrointestinal stromal tumours: recurrence patterns and prognostic factors for survival. Ann. Surg., 2000; 231: 51–58.

21. Roberts, P., Eisenberg, B. Clinical presentation of gastrointestinal stromal tumours and treatment of operable disease. Eur. J. Cancer, 2002; 38: S37–38.

22. Rutkowski, P., Debiec-Rychter, M., Nowecki, Z. I., et al. Different factors are responsible for predicting relapses after primary tumors resection and for imatinib treatment outcomes in gastrointestinal stromal tumors. Med. Sci. monit., 2007; 13: CR515–522.

23. Hassan, I., You, Y. N., Shyyan, R., et al. Surgically managed gastrointestinal stromal tumors: a komparative and prognostic analysis. Ann. Surg. Oncol., 2007; 15: 52–59.

24. Eilber, F. C., Rosen, G., Foscher, C., et al. Recurrent gastrointestinal stromal sarcomas. Surg. Oncol., 2000; 9: 71–75.

25. Heinrich, M. C., Griffith, D. J., Druker, B. J., et al. Inhibition of c-kit receptor tyrosine kinase aktivity by STI 571, a selective tyrosine kinase inhibitor. Blood, 2000; 96: 925–932.

26. Demetri, G. D. Identification and treatment of chemoresistant inoperable or metastatic GIST: experience with the selective tyrosin kinase inhibitor imatinib mesilate (STI 571). Eur. J. Cancer, 2002; 28: S52–59.

27. Demetri, G. D., von Mehren, M., Blanke, C. D., et al. Efficiacy and safety of imatinib mesilate in advanced gastrointestinal stromal tumours. N. Engl. J. Med., 2002; 347: 472–480.

28. De Matteo, R., Owzar, K., Maki, R., et al. Adjuvant imatinib mesylate increases recurrence free survival (RFS) in patients with completely resected localized primary gastrointestinal stromal tumor (GIST): North American Intergroup Phase III trial ACOSOG Z9001. Proc. Am. Soc. Clin. Oncol., 2007; Abstract No 10079.

29. Raut, C. P., De Matteo, R. P. Prognostic Factors for primary GIST: prime time for pesonalized therapy? Ann. Surg. Oncol., 2007; 15: 4–6.

30. Heindrich, M. C., Maki, R. G., Corless, C. L., et al. Sunitinib response in imatinib-resistant GIST correlates with KIT and PDGFR mutation status. J. Clin. Oncol., 2006; 24(Suppl): 9502.

31. Demetri, G. D., van Oosterom, A. T., Garrett, C. R., et al. Efficacy and safety of sunitinib in patients with advanced gastointestinal stromal tumor after failure of imatinib: a randomised controlled trial. Lancet, 2006; 368: 1329–1338.

32. Hirota, S., Isozaki, K., Moriyama, Y., et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science, 1998; 279: 577–580.

Surgery Orthopaedics Trauma surgery

Article was published in

Perspectives in Surgery

Issue 11

2009 Issue 11

Most read in this issue

This topic is also in:

Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account