J Korean Med Sci.  2010 Nov;25(11):1543-1552. 10.3346/jkms.2010.25.11.1543.

Clinical Practice Guideline for Accurate Diagnosis and Effective Treatment of Gastrointestinal Stromal Tumor in Korea

Affiliations
  • 1Department of Oncology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. ykkang@amc.seoul.kr
  • 2Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 3Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 4Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 5Division of Hematology/Oncology, Department of Internal Medicine, Korea Cancer Center Hospital, Seoul, Korea.
  • 6Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
  • 7Department of Surgery & Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Despite the rarity in incidence and prevalence, gastrointestinal stromal tumor (GIST) has emerged as a distinct pathogenetic entity. And the clinical management of GIST has been evolving very rapidly due to the recent recognition of its oncogenic signal transduction pathway and the introduction of new molecular-targeted therapy. Successful management of GIST requires a multidisciplinary approach firmly based on accurate histopathologic diagnosis. However, there was no standardized guideline for the management of Korean GIST patients. In 2007, the Korean GIST study group (KGSG) published the first guideline for optimal diagnosis and treatment of GIST in Korea. As the second version of the guideline, we herein have updated recent clinical recommendations and reflected changes in diagnosis, surgical and medical treatments for more optimal clinical practice for GIST in Korea. We hope the guideline can be of help in enhancing the quality of diagnosis by members of the Korean associate of physicians involving in GIST patients's care and subsequently in achieving optimal efficacy of treatment.

Keyword

Guideline; Gastrointestinal Stromal Tumors; Diagnosis; Surgery; Treatment; Chemotherapy, Targeted; Imatinib; Sunitinib

MeSH Terms

Antineoplastic Agents/therapeutic use
Gastrointestinal Stromal Tumors/*pathology/surgery/*therapy
Humans
Neoadjuvant Therapy
Piperazines/therapeutic use
Proto-Oncogene Proteins c-kit/metabolism
Pyrimidines/therapeutic use
Recurrence
Republic of Korea
Tumor Markers, Biological/metabolism

Figure

  • Fig. 1 Typical photomicrograph of spindle cell gastrointestinal stromal tumor (A: H&E, ×400) and c-kit stained in the cytoplasm and cytoplasmic membranes (B: Immunohistochemical stain, ×400). Blood vessels within the tumor are negative for c-kit (B).

  • Fig. 2 Typical photomicrograph of epithelioid gastrointestinal stromal tumor (A: H&E, ×400) and c-kit stained in the cytoplasm and cytoplasmic membranes (B: Immunohistochemical stain, ×400).

  • Fig. 3 Algorithm to diagnose gastrointestinal stromal tumor based on immunohistochemistry.


Cited by  3 articles

Long-Term Outcomes after Endoscopic Treatment of Gastric Gastrointestinal Stromal Tumor
Jong-Jae Park
Clin Endosc. 2016;49(3):232-234.    doi: 10.5946/ce.2016.052.

Standardization of the pathologic diagnosis of appendiceal mucinous neoplasms
Dong-Wook Kang, Baek-hui Kim, Joon Mee Kim, Jihun Kim, Hee Jin Chang, Mee Soo Chang, Jin-Hee Sohn, Mee-Yon Cho, So-Young Jin, Hee Kyung Chang, Hye Seung Han, Jung Yeon Kim, Hee Sung Kim, Do Youn Park, Ha Young Park, So Jeong Lee, Wonae Lee, Hye Seung Lee, Yoo Na Kang, Younghee Choi
J Pathol Transl Med. 2021;55(4):247-264.    doi: 10.4132/jptm.2021.05.28.

Clinical Practice Guideline for Accurate Diagnosis and Effective Treatment of Gastrointestinal Stromal Tumor in Korea
Yoon-Koo Kang, Hye Jin Kang, Kyoung-Mee Kim, Taesung Sohn, Dongil Choi, Min-Hee Ryu, Woo Ho Kim, Han-Kwang Yang,
Cancer Res Treat. 2012;44(2):85-96.    doi: 10.4143/crt.2012.44.2.85.


Reference

1. Casali PG, Jost L, Reichardt P, Schlemmer M, Blay JY. Gastrointestinal stromal tumours: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol. 2009. 20:Suppl 4. 64–67.
Article
2. Zalcberg JR, Desai J, Mann B, Fox S, Goldstein D, Mcarthur G, Clark M, Yip D. Consensus approaches to best practice management of gastrointestinal stromal tumors. Asia Pac J Clin Oncol. 2008. 4:188–198.
Article
3. Nishida T, Hirota S, Yanagisawa A, Sugino Y, Minami M, Yamamura Y, Otani Y, Shimada Y, Takahashi F, Kubota T. GIST Guideline Subcommittee. Clinical practice guidelines for gastrointestinal stromal tumor (GIST) in Japan: English version. Int J Clin Oncol. 2008. 13:416–430.
Article
4. Demetri GD, Antonia S, Benjamin RS, Bui MM, Casper ES, Conrad EU III, DeLaney TF, Ganjoo N, Kech R Jr, Heslin MJ, Hutchinson RJ, Kane JM III, Letson GD, McGarry SV, O'Donnell RJ, Paz IB, Pfeifer JD, Pollock RE, Randall RL, Riedel RF, Schupak DD, Schwartz HS, Thomton K, von Mehren M, Wayne JD. National Comprehensive Cancer Network Soft Tissue Sarcoma Panel. National Comprehensive Cancer Network Practice Guidelines in Oncology: Soft Tissue Sarcoma. J Natl Compr Canc Netw. 2010. Available at http://www.nccn.org/professionals/physician_gls/f_guidelines.asp.
5. Kang YK. Clinical practice guideline for adequate diagnosis and effective treatment of gastrointestinal stromal tumor in Korea. J Korean Med Assoc. 2007. 50:830–841.
Article
6. Kang DY, Park CK, Choi JS, Jin SY, Kim HJ, Joo M, Kang MS, Moon WS, Yun KJ, Yu ES, Kang H, Kim KM. Multiple gastrointestinal stromal tumors: Clinicopathologic and genetic analysis of 12 patients. Am J Surg Pathol. 2007. 31:224–232.
Article
7. Rubin BP, Heinrich MC, Corless CL. Gastrointestinal stromal tumour. Lancet. 2007. 369:1731–1741.
Article
8. Demetri GD, Benjamin RS, Blanke CD, Blay JY, Casali P, Choi H, Corless CL, Debiec-Rychter M, DeMatteo RP, Ettinger DS, Fisher GA, Fletcher CD, Gronchi A, Hohenberger P, Hughes M, Joensuu H, Judson I, Le Cesne A, Maki RG, Morse M, Pappo AS, Pisters PW, Raut CP, Reichardt P, Tyler DS, Van den Abbeele AD, von Mehren M, Wayne JD, Zalcberg J. NCCN Task Force. NCCN Task Force report: management of patients with gastrointestinal stromal tumor (GIST)--update of the NCCN clinical practice guidelines. J Natl Compr Canc Netw. 2007. 5:Suppl 2. S1–S29.
Article
9. Heinrich MC, Corless CL, Demetri GD, Blanke CD, von Mehren M, Joensuu H, McGreevey LS, Chen CJ, Van den Abbeele AD, Druker BJ, Kiese B, Eisenberg B, Roberts PJ, Singer S, Fletcher CD, Silberman S, Dimitrijevic S, Fletcher JA. Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol. 2003. 21:4342–4349.
Article
10. Kim KM, Kang DW, Moon WS, Park JB, Park CK, Sohn JH, Jeong JS, Cho MY, Jin SY, Choi JS, Kang DY. Gastrointestinal Stromal Tumor Committee. Korean Gastrointestinal Pathology Study Group. Gastrointestinal stromal tumors in Koreans: it's incidence and the clinical, pathologic and immunohistochemical findings. J Korean Med Sci. 2005. 20:977–984.
Article
11. Cho MY, Sohn JH, Kim JM, Kim KM, Park YS, Kim WH, Jung JS, Jung ES, Jin SY, Kang DY, Park JB, Park HS, Choi YD, Sung SH, Kim YB, Kim H, Bae YK, Kang M, Chang HJ, Chae YS, Lee HE, Park do Y, Lee YS, Kang YK, Kim HK, Chang HK, Hong SW, Choi YH, Shin O, Gu M, Kim YW, Kim GI, Chang SJ. Current trends in the epidemiological and pathological characteristics of gastrointestinal stromal tumors in Korea, 2003-2004. J Korean Med Sci. 2010. 25:853–862.
Article
12. Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006. 130:1466–1478.
Article
13. 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. 33:459–465.
Article
14. Liegl B, Hornick JL, Lazar AJ. Contemporary pathology of gastrointestinal stromal tumors. Hematol Oncol Clin North Am. 2009. 23:49–68.
Article
15. Kim KM, Kang DW, Moon WS, Park JB, Park CK, Sohn JH, Jeong JS, Cho MY, Jin SY, Choi JS, Kang DY. PKCtheta expression in gastrointestinal stromal tumor. Mod Pathol. 2006. 19:1480–1486.
16. Liegl B, Hornick JL, Corless CL, Fletcher CD. Monoclonal antibody DOG1.1 shows higher sensitivity than KIT in the diagnosis of gastrointestinal stromal tumors, including unusual subtypes. Am J Surg Pathol. 2009. 33:437–446.
Article
17. Lee HE, Kim MA, Lee HS, Lee BL, Kim WH. Characteristics of KIT-negative gastrointestinal stromal tumours and diagnostic utility of protein kinase C theta immunostaining. J Clin Pathol. 2008. 61:722–729.
Article
18. Orosz Z, Tornoczky T, Sapi Z. Gastrointestinal stromal tumors: a clinicopathologic and immunohistochemical study of 136 cases. Pathol Oncol Res. 2005. 11:11–21.
Article
19. Medeiros F, Corless CL, Duensing A, Hornick JL, Oliveira AM, Heinrich MC, Fletcher JA, Fletcher CD. KIT-negative gastrointestinal stromal tumors: proof of concept and therapeutic implications. Am J Surg Pathol. 2004. 28:889–894.
20. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol. 2006. 23:70–83.
Article
21. Blay JY, Bonvalot S, Casali P, Choi H, Debiec-Richter M, Dei Tos AP, Emile JF, Gronchi A, Hogendoorn PC, Joensuu H, Le Cesne A, McClure J, Maurel J, Nupponen N, Ray-Coquard I, Reichardt P, Sciot R, Stroobants S, van Glabbeke M, van Oosterom A, Demetri GD. GIST consensus meeting panelists. Consensus meeting for the management of gastrointestinal stromal tumors. Report of the GIST Consensus Conference of 20-21 March 2004, under the auspices of ESMO. Ann Oncol. 2005. 16:566–578.
Article
22. Schwartz SI, Shires GT. Schwartz's principles of surgery. 2005. 8th ed. New York: McGraw-Hill.
23. Townsend CM, Sabiston DC. Sabiston textbook of surgery: The biological basis of modern surgical practice. 2004. 17th ed. Philadelphia: W.B. Saunders Co..
24. Trent JC, Benjamin RS. New developments in gastrointestinal stromal tumor. Curr Opin Oncol. 2006. 18:386–395.
Article
25. Ryu MH, Lee JL, Chang HM, Kim TW, Kang HJ, Sohn HJ, Lee JS, Kang YK. Patterns of progression in gastrointestinal stromal tumor treated with imatinib mesylate. Jpn J Clin Oncol. 2006. 36:17–24.
Article
26. Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg. 2006. 244:176–184.
27. Iwahashi M, Takifuji K, Ojima T, Nakamura M, Nakamori M, Nakatani Y, Ueda K, Ishida K, Naka T, Ono K, Yamaue H. Surgical management of small gastrointestinal stromal tumors of the stomach. World J Surg. 2006. 30:28–35.
Article
28. Choi SM, Kim MC, Jung GJ, Kim HH, Kwon HC, Choi SR, Jang JS, Jeong JS. Laparoscopic wedge resection for gastric GIST: long-term follow-up results. Eur J Surg Oncol. 2007. 33:444–447.
Article
29. Novitsky YW, Kercher KW, Sing RF, Heniford BT. Long-term outcomes of laparoscopic resection of gastric gastrointestinal stromal tumors. Ann Surg. 2006. 243:738–745.
Article
30. Otani Y, Furukawa T, Yoshida M, Saikawa Y, Wada N, Ueda M, Kubota T, Mukai M, Kameyama K, Sugino Y, Kumai K, Kitajima M. Operative indications for relatively small (2-5 cm) gastrointestinal stromal tumor of the stomach based on analysis of 60 operated cases. Surgery. 2006. 139:484–492.
Article
31. Pister PW, Blay JY. GIST Guidelines: from Evidence to Practice, Evolving Consensus in GIST Management Meeting Monograph. 2007. In : GIST Gloval Opinion Leader Summit; Geneva.
32. 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. 231:51–58.
33. Raut CP, Posner M, Desai J, Morgan JA, George S, Zahrieh D, Fletcher CD, Demetri GD, Bertagnolli MM. Surgical management of advanced gastrointestinal stromal tumors after treatment with targeted systemic therapy using kinase inhibitors. J Clin Oncol. 2006. 24:2325–2331.
Article
34. Sym SJ, Ryu MH, Lee JL, Chang HM, Kim TW, Kim HC, Kim KH, Yook JH, Kim BS, Kang YK. Surgical intervention following imatinib treatment in patients with advanced gastrointestinal stromal tumors (GISTs). J Surg Oncol. 2008. 98:27–33.
Article
35. Dematteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD, Blackstein ME, Blanke CD, von Mehren M, Brennan MF, Patel S, McCarter MD, Polikoff JA, Tan BR, Owzar K. American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team. Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo-controlled trial. Lancet. 2009. 373:1097–1104.
Article
36. Kang B, Lee J, Ryu M, Im S, Park S, Kang W, Kim T, Oh D, Jung K, Kang Y. A phase II study of imatinib mesylate as adjuvant treatment for curatively resected high-risk localized gastrointestinal stromal tumors. J Clin Oncol. 2009. 27:Suppl. abstr e21515.
Article
37. Kim TW, Ryu MH, Lee H, Sym SJ, Lee JL, Chang HM, Park YS, Lee KH, Kang WK, Shin DB, Bang YJ, Lee JS, Kang YK. Kinase mutations and efficacy of imatinib in Korean patients with advanced gastrointestinal stromal tumors. Oncologist. 2009. 14:540–547.
Article
38. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008. 39:1411–1419.
Article
39. Bumming P, Andersson J, Meis-Kindblom JM, Klingenstierna H, Engstrom K, Stierner U, Wangberg B, Jansson S, Ahlman H, Kindblom LG, Nilsson B. Neoadjuvant, adjuvant and palliative treatment of gastrointestinal stromal tumours (GIST) with imatinib: a centre-based study of 17 patients. Br J Cancer. 2003. 89:460–464.
Article
40. Loughrey MB, Mitchell C, Mann GB, Michael M, Waring PM. Gastrointestinal stromal tumour treated with neoadjuvant imatinib. J Clin Pathol. 2005. 58:779–781.
Article
41. Oh JS, Lee JL, Kim MJ, Ryu MH, Chang HM, Kim TW, Jang SJ, Yook JH, Oh ST, Kim BS, Kang YK. Neoadjuvant imatinib in locally advanced gastrointestinal stromal tumors of stomach: Report of three cases. Cancer Res Treat. 2006. 38:178–183.
42. Dematteo RP, Heinrich MC, El-Rifai WM, Demetri G. Clinical management of gastrointestinal stromal tumors: before and after STI-571. Hum Pathol. 2002. 33:466–477.
Article
43. Rankin C, von Mehren M, Blanke C, Benjamin R, Fletcher CD, Bramwell V, Crowley J, Borden E, Demetri GD. Dose effect of imatinib (im) in patients (pts) with metastatic GIST - Phase III Sarcoma Group Study S0033. J Clin Oncol. 2004. 22:abstr 9005.
Article
44. Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, Issels R, van Oosterom A, Hogendoorn PC, Van Glabbeke M, Bertulli R, Judson I. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004. 364:1127–1134.
Article
45. Debiec-Rychter M, Sciot R, Le Cesne A, Schlemmer M, Hohenberger P, van Oosterom AT, Blay JY, Leyvraz S, Stul M, Casali PG, Zalcberg J, Verweij J, Van Glabbeke M, Hagemeijer A, Judson I. EORTC Soft Tissue and Bone Sarcoma Group. Italian Sarcoma Group. Australasian GastroIntestinal Trials Group. KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumours. Eur J Cancer. 2006. 42:1093–1103.
Article
46. Antoch G, Kanja J, Bauer S, Kuehl H, Renzing-Koehler K, Schuette J, Bockisch A, Debatin JF, Freudenberg LS. Comparison of PET, CT, and dual-modality PET/CT imaging for monitoring of imatinib (STI571) therapy in patients with gastrointestinal stromal tumors. J Nucl Med. 2004. 45:357–365.
47. Jager PL, Gietema JA, van der Graaf WT. Imatinib mesylate for the treatment of gastrointestinal stromal tumours: best monitored with FDG PET. Nucl Med Commun. 2004. 25:433–438.
Article
48. Stroobants S, Goeminne J, Seegers M, Dimitrijevic S, Dupont P, Nuyts J, Martens M, van den Borne B, Cole P, Sciot R, Dumez H, Silberman S, Mortelmans L, van Oosterom A. 18FDG-Positron emission tomography for the early prediction of response in advanced soft tissue sarcoma treated with imatinib mesylate (Glivec). Eur J Cancer. 2003. 39:2012–2020.
Article
49. Choi H, Charnsangavej C, de Castro Faria S, Tamm EP, Benjamin RS, Johnson MM, Macapinlac HA, Podoloff DA. CT evaluation of the response of gastrointestinal stromal tumors after imatinib mesylate treatment: a quantitative analysis correlated with FDG PET findings. AJR Am J Roentgenol. 2004. 183:1619–1628.
50. Choi H, Charnsangavej C, Faria SC, Macapinlac HA, Burgess MA, Patel SR, Chen LL, Podoloff DA, Benjamin RS. Correlation of computed tomography and positron emission tomography in patients with metastatic gastrointestinal stromal tumor treated at a single institution with imatinib mesylate: proposal of new computed tomography response criteria. J Clin Oncol. 2007. 25:1753–1759.
Article
51. Linton KM, Taylor MB, Radford JA. Response evaluation in gastrointestinal stromal tumours treated with imatinib: Misdiagnosis of disease progression on CT due to cystic change in liver metastases. Br J Radiol. 2006. 79:e40–e44.
Article
52. Desai J, Shankar S, Heinrich MC, Fletcher JA, Fletcher CD, Tuncali K, Silverman SG, Van Den Abbeele AD, Vansonnenberg E, Demetri GD. Clonal evolution of resistance to imatinib (IM) in patients (pts) with gastrointestinal stromal tumor (GIST): Molecular and radiologic evaluation of new lesions. J Clin Oncol. 2004. 22:abstr 3010.
Article
53. Bauer S, Hartmann JT, de Wit M, Lang H, Grabellus F, Antoch G, Niebel W, Erhard J, Ebeling P, Zeth M, Taeger G, Seeber S, Flasshove M, Schütte J. Resection of residual disease in patients with metastatic gastrointestinal stromal tumors responding to treatment with imatinib. Int J Cancer. 2005. 117:316–325.
Article
54. Zalcberg JR, Verweij J, Casali PG, Le Cesne A, Reichardt P, Blay JY, Schlemmer M, Van Glabbeke M, Brown M, Judson IR. EORTC Soft Tissue and Bone Sarcoma Group, the Italian Sarcoma Group. Australasian Gastrointestinal Trials Group. Outcome of patients with advanced gastro-intestinal stromal tumours crossing over to a daily imatinib dose of 800 mg after progression on 400 mg. Eur J Cancer. 2005. 41:1751–1757.
55. Park I, Ryu MH, Sym SJ, Lee SS, Jang G, Kim TW, Chang HM, Lee JL, Lee H, Kang YK. Dose escalation of imatinib after failure of standard dose in Korean patients with metastatic or unresectable gastrointestinal stromal tumor. Jpn J Clin Oncol. 2009. 39:105–110.
Article
56. Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, McArthur G, Judson IR, Heinrich MC, Morgan JA, Desai J, Fletcher CD, George S, Bello CL, Huang X, Baum CM, Casali PG. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006. 368:1329–1338.
Article
Full Text Links
  • JKMS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr