Treatment of gastrointestinal stromal tumors – a comprehensive view of the surgeon
Authors:
L. Fiala 1; R. Šefr 1; I. Kocáková 2; M. Pacal 3
Authors‘ workplace:
Oddělení chirurgické onkologie a centrálních operačních sálů, Masarykův onkologický ústav Brno, primář: MUDr. V. Chrenko, CSc.
1; Klinika komplexní onkologické péče, Masarykův onkologický ústav Brno, přednosta: prof. MUDr. R. Vyzula, CSc.
2; Oddělení radiologie, Masarykův onkologický ústav Brno, primář: MUDr. H. Bartoňková
3
Published in:
Rozhl. Chir., 2015, roč. 94, č. 5, s. 189-192.
Category:
Review
Overview
Introduction:
Gastrointestinal stromal tumors (GIST) are specific mesenchymal tumors of the gastrointestinal tract. Most of GISTs (95%) result from activating mutations in one of the receptor tyrosine kinase proteins (KIT). Tumor cells express this protein. GIST is most common in the stomach and small intestine, but may occur anywhere in the gastrointestinal tract and intra-abdominal soft tissues. The variety of its clinical presentations is related to localization of the tumor, its size and relationship to surrounding organs. Surgery is the first choice of treatment for patients with localized or potentially resectable tumors with the intention of R0 resection. Targeted therapy with imatinib (a selective inhibitor of the KIT protein) is the primary option for patients with metastatic GIST, as adjuvant treatment after surgery or neo-adjuvant therapy prior to surgery in indicated cases. This paper describes comprehensive therapy of GIST with an emphasis laid on the status of surgical treatment, and it highlights some controversial topics, e.g. the role of surgery for metastatic disease or neo-adjuvant targeted therapy.
Conclusion:
GIST is a relatively rare tumor most commonly affecting the stomach and small intestine. Surgical treatment is not replaceable in the treatment strategy of this disease. The diagnostic-therapeutic approach to patients with GIST must be comprehensive. Due to prescribing restrictions of biological therapies, these patients are inevitably directed to specialized centers where surgical treatment should also be preferably provided.
Key words:
GIST – gastroinestinal stromal tumor – imatinib
Sources
1. Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 2006;130(10):1466–78.
2. Bednarski BK, Pisters PWT, Hunt KK. The role of surgery in the multidisciplinary management of patients with localized gastrointestinal stromal tumors. Expert Rev Anticancer Ther 2012;12:1069–78.
3. Martin JF, Bazin P, Feroldi J, et al. Intramural myoid tumor of the stomach. Microscopic considerations on 6 cases. Ann Anat Pathol 1960;5:484−97.
4. Páral J, Lochman P, Kalábová H, et al. GIST: Novodobé poznatky a léčebné modality. Rozhl Chir 2012;4:189−98.
5. Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol 1983;7:507−19.
6. Nishida T, Hirota S, Taniguchi M, et al. Familial gastrointestinal stromal tumours with germline mutation of the KIT gene. Nat Genet 1998;19:323−24.
7. Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998;279:577−80.
8. von Mehren M, Benjamin RS, Bui MM, et al. NCCN Clinical Practice Guidelines in Oncology: Soft Tissue Sarcoma. Version 2, 2012. Dostupný z www: http://www.gistonline.it/Portali/1/Documents/NCCN Soft Tissue Sarcoma v.3.2012.pdf.
9. Heinrich MC, Corless CL, Duensing A, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science 2003;299:708–10.
10. Nilsson B, Bumming P, Medis-Kindblom JM, et al. Gastrointestinal stromal tumors: The incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era − a population-based study in western Sweden. Cancer 2005;103:821–9.
11. Miettinen M, Lasota J. Histopathology of Gastrointestinal Stromal Tumor. Journal of Surgical Oncology 2011;104:865–73.
12. Neuhaus SJ, Clark MA, Hayes AJ, et al. Surgery for Gastrointestinal stromal tumour in the post-imatinib era, ANZ J Surg 2005;75:165–72.
13. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002;33:459–65.
14. Cao H, Zhang Y, Wang M, et al. Prognostic analysis of patients with gastrointestinal stromal tumors: a single unit experience with surgical treatment of primary disease, Chinese Medical Journal 2010;123:131−6.
15. Miettinen M, Makhlouf HR, Sobin LH, et al. Gastrointestinal stromal tumors (GISTs) of the jejunum and ileum: a clinicopathologic, immunohistochemical and molecular genetic study of 906 cases prior to imatinib with longterm follow-up. Am J Surg Pathol 2006;30:477–89.
16. Wente MN, Büchler MW, Weitz J. Gastrointestinale Stromatumoren (GIST). Chirurgische Therapie, Chirurg 2008;79: 638–43.
17. Blanke CD, Demetri GD, von Mehren M, et al. Long-term results from a randomized phase II trial of standard-versus higher-dose imatinib mesylate for patients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J Clin Oncol 2008;26:620–5.
18. Kocakova I, Kocak I, Spelda S, et al. Long term experience of one center with targeted therapies consisting of imatinib mesylate and sunitinib malate in patients with unresectable or metastatic KIT positive gastrointestinal stromal tumors. Bratislava Medical Journal 2015;4:218−21.
19. Raut PCh, Gronchi A. Cytoreductive Surgery in Advanced GIST: Timing is Everything, Ann Surg Oncol 2013;20:4059–60.
20. Fiore M, Palassini E, Fumagalli E, et al. Preoperative imatinib mesylate for unresectable or locally advanced primary gastrointestinal stromal tumors (GIST). Eur J Surg Oncol 2009;35:739−45.
21. Blesius A, Cassier PA, Bertucci F, et al. Neoadjuvant imatinib in patients with locally advanced non metastatic GIST in the prospective BFR14 trial. BMC Cancer 2011;11:72.
Labels
Surgery Orthopaedics Trauma surgeryArticle was published in
Perspectives in Surgery
2015 Issue 5
Most read in this issue
- Pancreatic metastases − diagnosis, radical surgery, complications and survival
- Intestinal volvulus caused by the ingestion of magnet balls: unexpected risk in children
- Endometriosis of the appendix presenting like acute appendicitis – a case report
- Femoropopliteal deep vein thrombosis and popliteal artery pseudoaneurysm as a complication of multiple hereditary osteochondromatosis