The Korean Journal of Medicine

  • 제85권4호
  • /
  • Pages.341-353
  • /
  • 2013
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  • 1738-9364(pISSN)
  • /
  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
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위장관기질종양의 진단과 치료

Diagnosis and Treatment of Gastrointestinal Stromal Tumor

  • 강윤구 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 구동회 (성균관대학교 의과대학 강북삼성병원 혈액종양내과)
  • Kang, Yoon-Koo (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Koo, Dong Hoe (Division of Hematology/Oncology, Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine)
  • 발행 : 2013.10.01
⟨ 이전 논문 다음 논문 ⟩

초록

Gastrointestinal stromal tumors (GISTs), which are the most common mesenchymal tumors of the gastrointestinal tract, have represented an important advance in oncology field with the success of molecular targeted therapy. Since the approval of the tyrosine kinase inhibitor (imatinib) in 2002, the survival of patients with advanced GISTs has significantly increased. Accurate histopathologic diagnosis of GISTs and multidisciplinary approach has become more important for successful management of GISTs. Recently, imatinib has become a standard treatment even in adjuvant setting, and regorafenib has been approved for advanced GIST after failure of imatinib and sunitinib. This review presents here the updated results of relevant clinical studies for the further revision to the guideline of Korean GIST study group. We hope this review will help enhance the quality of diagnosis, treatment, and care of patients with GIST in Korea.

키워드

참고문헌

  1. Kang YK, Kang HJ, Kim KM, et al. Clinical practice guideline for accurate diagnosis and effective treatment of gastrointestinal stromal tumor in Korea. Cancer Res Treat 2012;44:85-96. https://doi.org/10.4143/crt.2012.44.2.85
  2. Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol 2004;22:3813-3825. https://doi.org/10.1200/JCO.2004.05.140
  3. 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. https://doi.org/10.1126/science.279.5350.577
  4. Heinrich MC, Corless CL, Duensing A, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science 2003;299:708-710. https://doi.org/10.1126/science.1079666
  5. Yarden Y, Kuang WJ, Yang-Feng T, et al. Human protooncogene c-kit: a new cell surface receptor tyrosine kinase for an unidentified ligand. EMBO J 1987;6:3341-3351.
  6. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-12. https://doi.org/10.1007/s004280000338
  7. Zöller ME, Rembeck B, Oden A, Samuelsson M, Angervall L. Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population. Cancer 1997;79:2125-2131. https://doi.org/10.1002/(SICI)1097-0142(19970601)79:11<2125::AID-CNCR9>3.0.CO;2-N
  8. Carney JA, Sheps SG, Go VL, Gordon H. The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. N Engl J Med 1977;296: 1517-1518. https://doi.org/10.1056/NEJM197706302962609
  9. Janeway KA, Kim SY, Lodish M, et al. Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations. Proc Natl Acad Sci U S A 2011;108:314-318. https://doi.org/10.1073/pnas.1009199108
  10. Carney JA, Stratakis CA. Familial paraganglioma and gastric stromal sarcoma: a new syndrome distinct from the Carney triad. Am J Med Genet 2002;108:132-139. https://doi.org/10.1002/ajmg.10235
  11. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002;33:459-465. https://doi.org/10.1053/hupa.2002.123545
  12. Liegl B, Hornick JL, Lazar AJ. Contemporary pathology of gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009;23:49-68. https://doi.org/10.1016/j.hoc.2008.12.002
  13. Kim KM, Kang DW, Moon WS, et al. PKCtheta expression in gastrointestinal stromal tumor. Mod Pathol 2006;19: 1480-1486.
  14. 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.
  15. Medeiros F, Corless CL, Duensing A, et al. KIT-negative gastrointestinal stromal tumors: proof of concept and therapeutic implications. Am J Surg Pathol 2004;28:889-894. https://doi.org/10.1097/00000478-200407000-00007
  16. Orosz Z, Tornoczky T, Sapi Z. Gastrointestinal stromal tumors: a clinicopathologic and immunohistochemical study of 136 cases. Pathol Oncol Res 2005;11:11-21. https://doi.org/10.1007/BF03032400
  17. Kang GH, Srivastava A, Kim YE, et al. DOG1 and PKC-$\Theta$ are useful in the diagnosis of KIT-negative gastrointestinal stromal tumors. Mod Pathol 2011;24:866-875. https://doi.org/10.1038/modpathol.2011.11
  18. Ryu MH, Lee JL, Chang HM, et al. Patterns of progression in gastrointestinal stromal tumor treated with imatinib mesylate. Jpn J Clin Oncol 2006;36:17-24. https://doi.org/10.1093/jjco/hyi212
  19. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006;23:70-83. https://doi.org/10.1053/j.semdp.2006.09.001
  20. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol 2008;39: 1411-1419. https://doi.org/10.1016/j.humpath.2008.06.025
  21. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Int J Surg Pathol. 2002;10:81-89. https://doi.org/10.1177/106689690201000201
  22. Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 2006;130: 1466-1478.
  23. Hornick JL, Fletcher CD. The role of KIT in the management of patients with gastrointestinal stromal tumors. Hum Pathol 2007;38:679-687. https://doi.org/10.1016/j.humpath.2007.03.001
  24. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999;30:1213-1220. https://doi.org/10.1016/S0046-8177(99)90040-0
  25. Blay JY, Bonvalot S, Casali P, et al. 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. https://doi.org/10.1093/annonc/mdi127
  26. Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg 2006;244:176-184. https://doi.org/10.1097/01.sla.0000218080.94145.cf
  27. Iwahashi M, Takifuji K, Ojima T, et al. Surgical management of small gastrointestinal stromal tumors of the stomach. World J Surg 2006;30:28-35. https://doi.org/10.1007/s00268-005-7944-4
  28. Kong SH, Yang HK. Surgical treatment of gastric gastrointestinal stromal tumor. J Gastric Cancer 2013;13:3-18. https://doi.org/10.5230/jgc.2013.13.1.3
  29. Choi SM, Kim MC, Jung GJ, et al. Laparoscopic wedge resection for gastric GIST: long-term follow-up results. Eur J Surg Oncol 2007;33:444-447. https://doi.org/10.1016/j.ejso.2006.11.003
  30. 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. https://doi.org/10.1097/01.sla.0000219739.11758.27
  31. Otani Y, Furukawa T, Yoshida M, et al. 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. https://doi.org/10.1016/j.surg.2005.08.011
  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. https://doi.org/10.1097/00000658-200001000-00008
  33. Raut CP, Posner M, Desai J, et al. Surgical management of advanced gastrointestinal stromal tumors after treatment with targeted systemic therapy using kinase inhibitors. J Clin Oncol 2006;24:2325-2331. https://doi.org/10.1200/JCO.2005.05.3439
  34. Sym SJ, Ryu MH, Lee JL, et al. Surgical intervention following imatinib treatment in patients with advanced gastrointestinal stromal tumors (GISTs). J Surg Oncol 2008;98:27-33. https://doi.org/10.1002/jso.21065
  35. Park SJ, Ryu M-H, Ryoo B-Y, Sohn BS, Kim K-H, Oh ST, et al. The role of surgical resection following imatinib treatment in patients with metastatic or recurrent GIST. J Clin Oncol 2013 Gastrointestinal Cancers Symposium Vol 31, No 4_suppl (Abstr 62).[Cited at 2013 June 15] Available from: http://meeting.ascopubs.org/cgi/content/abstract/31/4_ suppl/62?sid=37590461-2c95-47a8-826a-308e8d8c5ad6.
  36. Hislop J, Mowatt G, Sharma P, et al. Systematic review of escalated imatinib doses compared with sunitinib or best supportive care, for the treatment of people with unresectable/metastatic gastrointestinal stromal tumours whose disease has progressed on the standard imatinib dose. J Gastrointest Cancer 2012;43:168-176.
  37. Dematteo RP, Ballman KV, Antonescu CR, et al. Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, doubleblind, placebo-controlled trial. Lancet 2009;373:1097-1104. https://doi.org/10.1016/S0140-6736(09)60500-6
  38. Joensuu H, Eriksson M, Sundby Hall K, et al. One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA 2012;307:1265- 1272. https://doi.org/10.1001/jama.2012.347
  39. Bümming P, Andersson J, Meis-Kindblom JM, et al. 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. https://doi.org/10.1038/sj.bjc.6600965
  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. https://doi.org/10.1136/jcp.2004.023226
  41. Oh JS, Lee JL, Kim MJ, et al. Neoadjuvant imatinib in locally advanced gastrointestinal stromal tumors of the stomach: report of three cases. Cancer Res Treat 2006;38: 178-183. https://doi.org/10.4143/crt.2006.38.3.178
  42. Liegl B, Hornick JL, Lazar AJ. Contemporary pathology of gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009;23:49-68. https://doi.org/10.1016/j.hoc.2008.12.002
  43. An HJ, Ryu M-H, Ryoo B-Y, Sohn BS, Kim K-H, Oh ST, et al. The role of surgical cytoreduction before imatinib therapy in patients with advanced GIST. J Clin Oncol 30, 2012 (suppl; abstr 10093) [Cited at 2013 June 15] Available from: http://meetinglibrary.asco.org/content/98129-114.
  44. 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. https://doi.org/10.1053/hupa.2002.124122
  45. Verweij J, Casali PG, Zalcberg J, et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet 2004;364:1127-1134. https://doi.org/10.1016/S0140-6736(04)17098-0
  46. Debiec-Rychter M, Sciot R, Le Cesne A, et al. KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumours. Eur J Cancer 2006;42:1093-1103. https://doi.org/10.1016/j.ejca.2006.01.030
  47. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: soft tissue sarcoma: version 3 [Internet]. Washington: National Comprehensive Cancer Network, c2012 [cited 2013 Jun 15]. Available from: http: //www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf.
  48. Kim TW, Ryu MH, Lee H, et al. Kinase mutations and efficacy of imatinib in Korean patients with advanced gastrointestinal stromal tumors. Oncologist 2009;14:540-547. https://doi.org/10.1634/theoncologist.2008-0145
  49. Du CY, Shi YQ, Zhou Y, Fu H, Zhao G. The analysis of status and clinical implication of KIT and PDGFRA mutations in gastrointestinal stromal tumor (GIST). J Surg Oncol 2008;98:175-178. https://doi.org/10.1002/jso.21104
  50. Yeh CN, Chen TW, Lee HL, et al. Kinase mutations and imatinib mesylate response for 64 Taiwanese with advanced GIST: preliminary experience from Chang Gung Memorial Hospital. Ann Surg Oncol 2007;14:1123-1128. https://doi.org/10.1245/s10434-006-9288-1
  51. Kang HJ, Ryu MH, Kim KM, et al. Imatinib efficacy by tumor genotype in Korean patients with advanced gastrointestinal stromal tumors (GIST): the Korean GIST Study Group (KGSG) Study. Acta Oncol 2012;51:528-536. https://doi.org/10.3109/0284186X.2011.636753
  52. Imatinib dose escalation to 800 mg/day in Korean patients with metastatic or unresectable GIST harboring KIT exon 9 mutation (NCT01541709). [Cited at 2013 June 15] Available from: http://clinicaltrials.gov/ct2/show/NCT01541709?term= NCT01541709&rank=1.
  53. Patrikidou A, Chabaud S, Ray-Coquard I, et al. Influence of imatinib interruption and rechallenge on the residual disease in patients with advanced GIST: results of the BFR14 prospective French Sarcoma Group randomised, phase III trial. Ann Oncol 2013;24:1087-1093. https://doi.org/10.1093/annonc/mds587
  54. Antoch G, Kanja J, Bauer S, et al. 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.
  55. 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. https://doi.org/10.1097/00006231-200405000-00002
  56. Stroobants S, Goeminne J, Seegers M, et al. 18FDGPositron 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. https://doi.org/10.1016/S0959-8049(03)00073-X
  57. Choi H, Charnsangavej C, de Castro Faria S, et al. 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. https://doi.org/10.2214/ajr.183.6.01831619
  58. Choi H, Charnsangavej C, Faria SC, et al. 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. https://doi.org/10.1200/JCO.2006.07.3049
  59. 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:e40-44. https://doi.org/10.1259/bjr/62872118
  60. Desai J, Shankar S, Heinrich MC, et al. Clonal evolution of resistance to imatinib in patients with metastatic gastrointestinal stromal tumors. Clin Cancer Res 2007;13(18 Pt 1):5398-5405. https://doi.org/10.1158/1078-0432.CCR-06-0858
  61. Bauer S, Hartmann JT, de Wit M, et al. Resection of residual disease in patients with metastatic gastrointestinal stromal tumors responding to treatment with imatinib. Int J Cancer 2005;117:316-325. https://doi.org/10.1002/ijc.21164
  62. Zalcberg JR, Verweij J, Casali PG, et al. 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. https://doi.org/10.1016/j.ejca.2005.04.034
  63. Park I, Ryu MH, Sym SJ, et al. 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.
  64. Demetri GD, van Oosterom AT, Garrett CR, et al. 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. https://doi.org/10.1016/S0140-6736(06)69446-4
  65. Demetri GD, Reichardt P, Kang YK, Blay JY, Rutkowski P, Gelderblom H, et al. Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (GRID): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 2013; 381:295-302. https://doi.org/10.1016/S0140-6736(12)61857-1
  66. Kang Y-K, Ryu M-H, Ryoo B-Y, Kim HJ, Lee JJ, Yoo C, et al. Randomized phase III trial of imatinib (IM) rechallenge versus placebo (PL) in patients (pts) with metastatic and/or unresectable gastrointestinal stromal tumor (GIST) after failure of at least both IM and sunitinib (SU): RIGHT study. J Clin Oncol 31, 2013 (suppl; abstr LBA10502).[Cited at 2013 June 15] Available from: http://meetinglibrary.asco. org/content/116140-132.

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