Clin Colon Rectal Surg 2018; 31(05): 295-300
DOI: 10.1055/s-0038-1642053
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Gastrointestinal Stromal Tumors of the Distal Gastrointestinal Tract

Katherine A. Kelley
1   Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health & Science University, Portland, Oregon
,
Raphael Byrne
1   Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health & Science University, Portland, Oregon
,
Kim C. Lu
1   Division of Gastrointestinal and General Surgery, Department of Surgery, Oregon Health & Science University, Portland, Oregon
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
04. September 2018 (online)

Abstract

Gastrointestinal stromal tumors (GISTs) are rare in occurrence, but comprise the most common mesenchymal tumors of the gastrointestinal tract and affect between 15 and 20 individuals per million per year. Due to recent advancements in molecular classification of these tumors, medical therapy has provided improved outcomes to a historically surgically managed disease. This review article briefly discusses the molecular characteristics, medical and surgical therapies, and future of GIST management.

 
  • References

  • 1 Crosby JA, Catton CN, Davis A. , et al. Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database. Ann Surg Oncol 2001; 8 (01) 50-59
  • 2 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 (01) 51-58
  • 3 Lewis JJ, Brennan MF. Soft tissue sarcomas. Curr Probl Surg 1996; 33 (10) 817-872
  • 4 Nishida T, Hirota S. Biological and clinical review of stromal tumors in the gastrointestinal tract. Histol Histopathol 2000; 15 (04) 1293-1301
  • 5 Raut CP, DeMatteo RP. Prognostic factors for primary GIST: prime time for personalized therapy?. Ann Surg Oncol 2008; 15 (01) 4-6
  • 6 Raut CP. Current Surgical Therapy, 11th ed. Philadelphia: Saunders; 2013: 96-103
  • 7 Scarpa M, Bertin M, Ruffolo C, Polese L, D'Amico DF, Angriman I. A systematic review on the clinical diagnosis of gastrointestinal stromal tumors. J Surg Oncol 2008; 98 (05) 384-392
  • 8 Hirota S, Isozaki K, Moriyama Y. , et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279 (5350): 577-580
  • 9 Reichardt P, Hogendoorn PC, Tamborini E. , et al. Gastrointestinal stromal tumors I: pathology, pathobiology, primary therapy, and surgical issues. Semin Oncol 2009; 36 (04) 290-301
  • 10 Heinrich MC, Corless CL, Duensing A. , et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science 2003; 299 (5607): 708-710
  • 11 Mason EF, Hornick JL. Succinate dehydrogenase deficiency is associated with decreased 5-hydroxymethylcytosine production in gastrointestinal stromal tumors: implications for mechanisms of tumorigenesis. Mod Pathol 2013; 26 (11) 1492-1497
  • 12 Janeway KA, Kim SY, Lodish M. , et al; NIH Pediatric and Wild-Type GIST Clinic. Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations. Proc Natl Acad Sci U S A 2011; 108 (01) 314-318
  • 13 Demetri GD, Benjamin RS, Blanke CD. , et al; 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. 02) S1-S29 , quiz S30
  • 14 Miranda C, Nucifora M, Molinari F. , et al. KRAS and BRAF mutations predict primary resistance to imatinib in gastrointestinal stromal tumors. Clin Cancer Res 2012; 18 (06) 1769-1776
  • 15 Agaram NP, Wong GC, Guo T. , et al. Novel V600E BRAF mutations in imatinib-naive and imatinib-resistant gastrointestinal stromal tumors. Genes Chromosomes Cancer 2008; 47 (10) 853-859
  • 16 Stratakis CA, Carney JA. The triad of paragangliomas, gastric stromal tumours and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications. J Intern Med 2009; 266 (01) 43-52
  • 17 Sarlomo-Rikala M, Kovatich AJ, Barusevicius A, Miettinen M. CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol 1998; 11 (08) 728-734
  • 18 Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD117 (KIT). Mod Pathol 2000; 13 (10) 1134-1142
  • 19 Espinosa I, Lee CH, Kim MK. , et al. A novel monoclonal antibody against DOG1 is a sensitive and specific marker for gastrointestinal stromal tumors. Am J Surg Pathol 2008; 32 (02) 210-218
  • 20 Zhao WY, Xu J, Wang M. , et al. Prognostic value of Ki67 index in gastrointestinal stromal tumors. Int J Clin Exp Pathol 2014; 7 (05) 2298-2304
  • 21 Nilsson B, Bümming P, Meis-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 (04) 821-829
  • 22 Roberts PJ, Eisenberg B. Clinical presentation of gastrointestinal stromal tumors and treatment of operable disease. Eur J Cancer 2002; 38 (Suppl. 05) S37-S38
  • 23 Bauer S, Corless CL, Heinrich MC. , et al. Response to imatinib mesylate of a gastrointestinal stromal tumor with very low expression of KIT. Cancer Chemother Pharmacol 2003; 51 (03) 261-265
  • 24 Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006; 23 (02) 70-83
  • 25 Raut CP, Morgan JA, Ashley SW. Current issues in gastrointestinal stromal tumors: incidence, molecular biology, and contemporary treatment of localized and advanced disease. Curr Opin Gastroenterol 2007; 23 (02) 149-158
  • 26 Stroszczynski C, Jost D, Reichardt P. , et al. Follow-up of gastro-intestinal stromal tumours (GIST) during treatment with imatinib mesylate by abdominal MRI. Eur Radiol 2005; 15 (12) 2448-2456
  • 27 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 (05) 433-438
  • 28 Changchien CR, Wu MC, Tasi WS. , et al. Evaluation of prognosis for malignant rectal gastrointestinal stromal tumor by clinical parameters and immunohistochemical staining. Dis Colon Rectum 2004; 47 (11) 1922-1929
  • 29 Nguyen SQ, Divino CM, Wang JL, Dikman SH. Laparoscopic management of gastrointestinal stromal tumors. Surg Endosc 2006; 20 (05) 713-716
  • 30 McCarter MD, Antonescu CR, Ballman KV. , et al; American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant Gist Study Team. Microscopically positive margins for primary gastrointestinal stromal tumors: analysis of risk factors and tumor recurrence. J Am Coll Surg 2012; 215 (01) 53-59 , discussion 59–60
  • 31 Druker BJ, Talpaz M, Resta DJ. , et al. Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia. N Engl J Med 2001; 344 (14) 1031-1037
  • 32 van Oosterom AT, Judson I, Verweij J. , et al; European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. Safety and efficacy of imatinib (STI571) in metastatic gastrointestinal stromal tumours: a phase I study. Lancet 2001; 358 (9291): 1421-1423
  • 33 Demetri GD, von Mehren M, Blanke CD. , et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002; 347 (07) 472-480
  • 34 Verweij J, Casali PG, Zalcberg J. , et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet 2004; 364 (9440): 1127-1134
  • 35 Blanke CD, Rankin C, Demetri GD. , et al. Phase III randomized, intergroup trial assessing imatinib mesylate at two dose levels in patients with unresectable or metastatic gastrointestinal stromal tumors expressing the kit receptor tyrosine kinase: S0033. J Clin Oncol 2008; 26 (04) 626-632
  • 36 Gastrointestinal Stromal Tumor Meta-Analysis Group (MetaGIST). Comparison of two doses of imatinib for the treatment of unresectable or metastatic gastrointestinal stromal tumors: a meta-analysis of 1,640 patients. J Clin Oncol 2010; 28 (07) 1247-1253
  • 37 Lee CK, Goldstein D, Gibbs E. , et al. Development and validation of prognostic nomograms for metastatic gastrointestinal stromal tumour treated with imatinib. Eur J Cancer 2015; 51 (07) 852-860
  • 38 Joensuu H, Vehtari A, Riihimäki J. , et al. Risk of recurrence of gastrointestinal stromal tumour after surgery: an analysis of pooled population-based cohorts. Lancet Oncol 2012; 13 (03) 265-274
  • 39 Fletcher CD, Berman JJ, Corless C. , et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002; 33 (05) 459-465
  • 40 Rutkowski P, Bylina E, Wozniak A. , et al. Validation of the Joensuu risk criteria for primary resectable gastrointestinal stromal tumour - the impact of tumour rupture on patient outcomes. Eur J Surg Oncol 2011; 37 (10) 890-896
  • 41 DeMatteo RP, Ballman KV, Antonescu CR. , et al. Placebo-controlled randomized trial of adjuvant imatinib mesylate following the resection of localized, primary gastrointestinal stromal tumor (GIST). Lancet 2009; 373 (9669): 1097-1104
  • 42 Casali PG, Le Cesne A, Poveda Velasco A. , et al. Time to definitive failure to the first tyrosine kinase inhibitor in localized GI stromal tumors treated with imatinib as an adjuvant: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Intergroup Randomized Trial in collaboration with the Australasian Gastro-Intestinal Trials Group, UNICANCER, French Sarcoma Group, Italian Sarcoma Group, and Spanish Group for Research on Sarcomas. J Clin Oncol 2015; 33 (36) 4276-4283
  • 43 Eisenberg BL, Harris J, Blanke CD. , et al. Phase II trial of neoadjuvant/adjuvant imatinib mesylate (IM) for advanced primary and metastatic/recurrent operable gastrointestinal stromal tumor (GIST): early results of RTOG 0132/ACRIN 6665. J Surg Oncol 2009; 99 (01) 42-47
  • 44 Wilkinson MJ, Fitzgerald JE, Strauss DC. , et al. Surgical treatment of gastrointestinal stromal tumour of the rectum in the era of imatinib. Br J Surg 2015; 102 (08) 965-971
  • 45 Bauer S, Rutkowski P, Hohenberger P. , et al. Long-term follow-up of patients with GIST undergoing metastasectomy in the era of imatinib -- analysis of prognostic factors (EORTC-STBSG collaborative study). Eur J Surg Oncol 2014; 40 (04) 412-419
  • 46 DeMatteo RP, Maki RG, Singer S, Gonen M, Brennan MF, Antonescu CR. Results of tyrosine kinase inhibitor therapy followed by surgical resection for metastatic gastrointestinal stromal tumor. Ann Surg 2007; 245 (03) 347-352
  • 47 Zhan WH, Wang PZ, Shao YF. , et al. [Efficacy and safety of adjuvant post-surgical therapy with imatinib in gastrointestinal stromal tumor patients with high risk of recurrence: interim analysis from a multicenter prospective clinical trial]. Chin J Gastrointestinal Surg 2006; 9 (05) 383-387
  • 48 DeMatteo RP, Ballman KV, Antonescu CR. , et al; American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team for the Alliance for Clinical Trials in Oncology. Long-term results of adjuvant imatinib mesylate in localized, high-risk, primary gastrointestinal stromal tumor: ACOSOG Z9000 (Alliance) intergroup phase 2 trial. Ann Surg 2013; 258 (03) 422-429
  • 49 Kang YK, Ryu MH, Yoo C. , et al. Resumption of imatinib to control metastatic or unresectable gastrointestinal stromal tumours after failure of imatinib and sunitinib (RIGHT): a randomised, placebo-controlled, phase 3 trial. Lancet Oncol 2013; 14 (12) 1175-1182
  • 50 Corless CL, Ballman KV, Antonescu CR. , et al. Pathologic and molecular features correlate with long-term outcome after adjuvant therapy of resected primary GI stromal tumor: the ACOSOG Z9001 trial.. J Clin Oncol 2014; 32 (15) 1563-1570