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Epidemiology, classification, clinical presentation, prognostic features, and diagnostic work-up of gastrointestinal stromal tumors (GIST)

Jeffrey Morgan, MD
Chandrajit P Raut, MD, MSc, FACS
Anette Duensing, MD
Vicki L Keedy, MD, MSCI
Section Editors
Kenneth K Tanabe, MD
Robert Maki, MD, PhD
Alberto S Pappo, MD
Deputy Editor
Diane MF Savarese, MD


Stromal or mesenchymal neoplasms affecting the gastrointestinal tract typically present as subepithelial neoplasms, and they are divided broadly into two groups. The most common group consists of gastrointestinal stromal tumors (GISTs). These neoplasms are most often located in the stomach and proximal small intestine, but they can occur in any portion of the alimentary tract and occasionally in the omentum, mesentery, and peritoneum [1-5]. GISTs are identified mainly by expression of the KIT protein and frequently harbor activating mutations in the KIT or platelet-derived growth factor receptor alpha (PDGFRA) genes.

A far less common group of mesenchymal gastrointestinal tract neoplasms is comprised of a spectrum of tumors that are identical to those that might arise in the soft tissues throughout the rest of the body. These include lipomas, liposarcomas, leiomyomas, true leiomyosarcomas, desmoid tumors, schwannomas, and peripheral nerve sheath tumors [6].

The epidemiology, classification, molecular pathogenesis, and diagnostic workup of localized GISTs will be reviewed here. Treatment for GISTs is discussed elsewhere. (See "Tyrosine kinase inhibitor therapy for advanced gastrointestinal stromal tumors" and "Adjuvant and neoadjuvant imatinib for gastrointestinal stromal tumors" and "Local treatment for gastrointestinal stromal tumors, leiomyomas, and leiomyosarcomas of the gastrointestinal tract".)


Despite the fact that GISTs are the most common nonepithelial neoplasms involving the gastrointestinal tract, mesenchymal tumors only constitute approximately 1 percent of primary gastrointestinal cancers [2,3]. While the incidence was difficult to assess in earlier years because of the lack of diagnostic criteria, studies over the past decade or so place this in the range of 7 to 15 cases per million population per year [7-14]. As an example, a Surveillance, Epidemiology, and End Results (SEER) analysis of histologically confirmed GISTs after the implementation of a GIST-specific histology code identified 6142 cases diagnosed between 2001 and 2011, with an incidence of 0.68 per 100,000. [10]. Moreover, a prospective study of the population in the French Rhone Alps region conducted in 2005 to 2007 reported a crude incidence of GIST of 11.2 per million per year [15].

It is important to note, however, that autopsy data suggest that the frequency of incidentally detected subcentimeter gastric GIST lesions may be much higher than previously estimated. Two studies examining consecutive autopsy specimens found small GISTs (sized <1 mm to 1 cm) in 22.5 to 35 percent of the cases, respectively [16,17]. All lesions shared typical GIST immunohistochemical (positive for KIT and CD34 expression) and molecular features (KIT or platelet-derived growth factor receptor alpha [PDGFRA] mutation in up to 50 percent of the cases for which sufficient DNA was available), and almost all lesions were located in the upper stomach. Some have suggested that given the relatively low annual incidence of clinically diagnosed GISTs, only a few microscopic tumors may grow to a clinically relevant size with malignant potential [15]. Further studies are needed to confirm these observations and to clarify the genetic events responsible for the transformation of microscopic GIST lesions into clinically relevant GISTs.

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Literature review current through: Nov 2017. | This topic last updated: Nov 06, 2017.
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  1. Rubin BP, Fletcher JA, Fletcher CD. Molecular Insights into the Histogenesis and Pathogenesis of Gastrointestinal Stromal Tumors. Int J Surg Pathol 2000; 8:5.
  2. Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001; 438:1.
  3. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999; 30:1213.
  4. Reith JD, Goldblum JR, Lyles RH, Weiss SW. Extragastrointestinal (soft tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome. Mod Pathol 2000; 13:577.
  5. 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.
  6. Atlas of Tumor Pathology: Tumors of the esophagus and stomach. Electronic fascicle v2.0b, Armed Forces Institute of Pathology, Washington DC.
  7. Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005; 100:162.
  8. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 2002; 33:459.
  9. US population data from the US Census bureau, 2008 www.census.gov/popest/states/NST-ann-est.html (Accessed on March 08, 2011).
  10. Ma GL, Murphy JD, Martinez ME, Sicklick JK. Epidemiology of gastrointestinal stromal tumors in the era of histology codes: results of a population-based study. Cancer Epidemiol Biomarkers Prev 2015; 24:298.
  11. 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; 103:821.
  12. Tryggvason G, Gíslason HG, Magnússon MK, Jónasson JG. Gastrointestinal stromal tumors in Iceland, 1990-2003: the icelandic GIST study, a population-based incidence and pathologic risk stratification study. Int J Cancer 2005; 117:289.
  13. Goettsch WG, Bos SD, Breekveldt-Postma N, et al. Incidence of gastrointestinal stromal tumours is underestimated: results of a nation-wide study. Eur J Cancer 2005; 41:2868.
  14. Tzen CY, Wang JH, Huang YJ, et al. Incidence of gastrointestinal stromal tumor: a retrospective study based on immunohistochemical and mutational analyses. Dig Dis Sci 2007; 52:792.
  15. Cassier PA, Ducimetière F, Lurkin A, et al. A prospective epidemiological study of new incident GISTs during two consecutive years in Rhône Alpes region: incidence and molecular distribution of GIST in a European region. Br J Cancer 2010; 103:165.
  16. Kawanowa K, Sakuma Y, Sakurai S, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol 2006; 37:1527.
  17. Agaimy A, Wünsch PH, Hofstaedter F, et al. Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. Am J Surg Pathol 2007; 31:113.
  18. Miettinen M, Fetsch JF, Sobin LH, Lasota J. Gastrointestinal stromal tumors in patients with neurofibromatosis 1: a clinicopathologic and molecular genetic study of 45 cases. Am J Surg Pathol 2006; 30:90.
  19. Mussi C, Schildhaus HU, Gronchi A, et al. Therapeutic consequences from molecular biology for gastrointestinal stromal tumor patients affected by neurofibromatosis type 1. Clin Cancer Res 2008; 14:4550.
  20. Maeyama H, Hidaka E, Ota H, et al. Familial gastrointestinal stromal tumor with hyperpigmentation: association with a germline mutation of the c-kit gene. Gastroenterology 2001; 120:210.
  21. Nishida T, Hirota S, Taniguchi M, et al. Familial gastrointestinal stromal tumours with germline mutation of the KIT gene. Nat Genet 1998; 19:323.
  22. Hirota S, Okazaki T, Kitamura Y, et al. Cause of familial and multiple gastrointestinal autonomic nerve tumors with hyperplasia of interstitial cells of Cajal is germline mutation of the c-kit gene. Am J Surg Pathol 2000; 24:326.
  23. Beghini A, Tibiletti MG, Roversi G, et al. Germline mutation in the juxtamembrane domain of the kit gene in a family with gastrointestinal stromal tumors and urticaria pigmentosa. Cancer 2001; 92:657.
  24. Hirota S, Nishida T, Isozaki K, et al. Familial gastrointestinal stromal tumors associated with dysphagia and novel type germline mutation of KIT gene. Gastroenterology 2002; 122:1493.
  25. Chompret A, Kannengiesser C, Barrois M, et al. PDGFRA germline mutation in a family with multiple cases of gastrointestinal stromal tumor. Gastroenterology 2004; 126:318.
  26. de Raedt T, Cools J, Debiec-Rychter M, et al. Intestinal neurofibromatosis is a subtype of familial GIST and results from a dominant activating mutation in PDGFRA. Gastroenterology 2006; 131:1907.
  27. Pasini B, Matyakhina L, Bei T, et al. Multiple gastrointestinal stromal and other tumors caused by platelet-derived growth factor receptor alpha gene mutations: a case associated with a germline V561D defect. J Clin Endocrinol Metab 2007; 92:3728.
  28. Ricci R, Martini M, Cenci T, et al. PDGFRA-mutant syndrome. Mod Pathol 2015; 28:954.
  29. Andersson J, Sihto H, Meis-Kindblom JM, et al. NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic, and genotypic characteristics. Am J Surg Pathol 2005; 29:1170.
  30. Scarpa M, Bertin M, Ruffolo C, et al. A systematic review on the clinical diagnosis of gastrointestinal stromal tumors. J Surg Oncol 2008; 98:384.
  31. Janeway KA, Pappo A. Treatment guidelines for gastrointestinal stromal tumors in children and young adults. J Pediatr Hematol Oncol 2012; 34 Suppl 2:S69.
  32. Miettinen M, Lasota J, Sobin LH. Gastrointestinal stromal tumors of the stomach in children and young adults: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases with long-term follow-up and review of the literature. Am J Surg Pathol 2005; 29:1373.
  33. Agaram NP, Laquaglia MP, Ustun B, et al. Molecular characterization of pediatric gastrointestinal stromal tumors. Clin Cancer Res 2008; 14:3204.
  34. Call J, Walentas CD, Eickhoff JC, Scherzer N. Survival of gastrointestinal stromal tumor patients in the imatinib era: life raft group observational registry. BMC Cancer 2012; 12:90.
  35. Pappo AS, Janeway KA. Pediatric gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009; 23:15.
  36. 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.
  37. 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; 266:43.
  38. Pasini B, McWhinney SR, Bei T, et al. Clinical and molecular genetics of patients with the Carney-Stratakis syndrome and germline mutations of the genes coding for the succinate dehydrogenase subunits SDHB, SDHC, and SDHD. Eur J Hum Genet 2008; 16:79.
  39. McWhinney SR, Pasini B, Stratakis CA, International Carney Triad and Carney-Stratakis Syndrome Consortium. Familial gastrointestinal stromal tumors and germ-line mutations. N Engl J Med 2007; 357:1054.
  40. Carney JA. Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc 1999; 74:543.
  41. Haller F, Moskalev EA, Faucz FR, et al. Aberrant DNA hypermethylation of SDHC: a novel mechanism of tumor development in Carney triad. Endocr Relat Cancer 2014; 21:567.
  42. Matyakhina L, Bei TA, McWhinney SR, et al. Genetics of carney triad: recurrent losses at chromosome 1 but lack of germline mutations in genes associated with paragangliomas and gastrointestinal stromal tumors. J Clin Endocrinol Metab 2007; 92:2938.
  43. Newman PL, Wadden C, Fletcher CD. Gastrointestinal stromal tumours: correlation of immunophenotype with clinicopathological features. J Pathol 1991; 164:107.
  44. 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; 13:1134.
  45. Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279:577.
  46. Besmer P, Murphy JE, George PC, et al. A new acute transforming feline retrovirus and relationship of its oncogene v-kit with the protein kinase gene family. Nature 1986; 320:415.
  47. Rubin BP, Singer S, Tsao C, et al. KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res 2001; 61:8118.
  48. Lasota J, Jasinski M, Sarlomo-Rikala M, Miettinen M. Mutations in exon 11 of c-Kit occur preferentially in malignant versus benign gastrointestinal stromal tumors and do not occur in leiomyomas or leiomyosarcomas. Am J Pathol 1999; 154:53.
  49. Hirota S, Nishida T, Isozaki K, et al. Gain-of-function mutation at the extracellular domain of KIT in gastrointestinal stromal tumours. J Pathol 2001; 193:505.
  50. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Int J Surg Pathol 2002; 10:81.
  51. Sakurai S, Fukasawa T, Chong JM, et al. C-kit gene abnormalities in gastrointestinal stromal tumors (tumors of interstitial cells of Cajal. Jpn J Cancer Res 1999; 90:1321.
  52. Wang L, Vargas H, French SW. Cellular origin of gastrointestinal stromal tumors: a study of 27 cases. Arch Pathol Lab Med 2000; 124:1471.
  53. Sircar K, Hewlett BR, Huizinga JD, et al. Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol 1999; 23:377.
  54. Broudy VC. Stem cell factor and hematopoiesis. Blood 1997; 90:1345.
  55. Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol 2001; 54:96.
  56. Théou-Anton N, Tabone S, Brouty-Boyé D, et al. Co expression of SCF and KIT in gastrointestinal stromal tumours (GISTs) suggests an autocrine/paracrine mechanism. Br J Cancer 2006; 94:1180.
  57. Janeway KA, Liegl B, Harlow A, et al. Pediatric KIT wild-type and platelet-derived growth factor receptor alpha-wild-type gastrointestinal stromal tumors share KIT activation but not mechanisms of genetic progression with adult gastrointestinal stromal tumors. Cancer Res 2007; 67:9084.
  58. Corless CL, Schroeder A, Griffith D, et al. PDGFRA mutations in gastrointestinal stromal tumors: frequency, spectrum and in vitro sensitivity to imatinib. J Clin Oncol 2005; 23:5357.
  59. Agaimy A, Märkl B, Arnholdt H, et al. Sporadic segmental Interstitial cell of cajal hyperplasia (microscopic GIST) with unusual diffuse longitudinal growth replacing the muscularis propria: differential diagnosis to hereditary GIST syndromes. Int J Clin Exp Pathol 2010; 3:549.
  60. Yamamoto H, Oda Y, Kawaguchi K, et al. c-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue). Am J Surg Pathol 2004; 28:479.
  61. Miettinen M, Monihan JM, Sarlomo-Rikala M, et al. 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.
  62. Heinrich MC, Corless CL, Duensing A, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science 2003; 299:708.
  63. Hirota S, Ohashi A, Nishida T, et al. Gain-of-function mutations of platelet-derived growth factor receptor alpha gene in gastrointestinal stromal tumors. Gastroenterology 2003; 125:660.
  64. Novelli M, Rossi S, Rodriguez-Justo M, et al. DOG1 and CD117 are the antibodies of choice in the diagnosis of gastrointestinal stromal tumours. Histopathology 2010; 57:259.
  65. Miettinen M, Wang ZF, Lasota J. DOG1 antibody in the differential diagnosis of gastrointestinal stromal tumors: a study of 1840 cases. Am J Surg Pathol 2009; 33:1401.
  66. 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.
  67. West RB, Corless CL, Chen X, et al. The novel marker, DOG1, is expressed ubiquitously in gastrointestinal stromal tumors irrespective of KIT or PDGFRA mutation status. Am J Pathol 2004; 165:107.
  68. Duensing A, Joseph NE, Medeiros F, et al. Protein Kinase C theta (PKCtheta) expression and constitutive activation in gastrointestinal stromal tumors (GISTs). Cancer Res 2004; 64:5127.
  69. Kang GH, Srivastava A, Kim YE, et al. DOG1 and PKC-θ are useful in the diagnosis of KIT-negative gastrointestinal stromal tumors. Mod Pathol 2011; 24:866.
  70. Lux ML, Rubin BP, Biase TL, et al. KIT extracellular and kinase domain mutations in gastrointestinal stromal tumors. Am J Pathol 2000; 156:791.
  71. Emile JF, Théou N, Tabone S, et al. Clinicopathologic, phenotypic, and genotypic characteristics of gastrointestinal mesenchymal tumors. Clin Gastroenterol Hepatol 2004; 2:597.
  72. Andersson J, Sjögren H, Meis-Kindblom JM, et al. The complexity of KIT gene mutations and chromosome rearrangements and their clinical correlation in gastrointestinal stromal (pacemaker cell) tumors. Am J Pathol 2002; 160:15.
  73. Corless CL, Barnett CM, Heinrich MC. Gastrointestinal stromal tumours: origin and molecular oncology. Nat Rev Cancer 2011; 11:865.
  74. Yamamoto H, Tobo T, Nakamori M, et al. Neurofibromatosis type 1-related gastrointestinal stromal tumors: a special reference to loss of heterozygosity at 14q and 22q. J Cancer Res Clin Oncol 2009; 135:791.
  75. Martín J, Poveda A, Llombart-Bosch A, et al. Deletions affecting codons 557-558 of the c-KIT gene indicate a poor prognosis in patients with completely resected gastrointestinal stromal tumors: a study by the Spanish Group for Sarcoma Research (GEIS). J Clin Oncol 2005; 23:6190.
  76. Gasparotto D, Rossi S, Polano M, et al. Quadruple-Negative GIST Is a Sentinel for Unrecognized Neurofibromatosis Type 1 Syndrome. Clin Cancer Res 2017; 23:273.
  77. Rege TA, Wagner AJ, Corless CL, et al. "Pediatric-type" gastrointestinal stromal tumors in adults: distinctive histology predicts genotype and clinical behavior. Am J Surg Pathol 2011; 35:495.
  78. Boikos SA, Pappo AS, Killian JK, et al. Molecular Subtypes of KIT/PDGFRA Wild-Type Gastrointestinal Stromal Tumors: A Report From the National Institutes of Health Gastrointestinal Stromal Tumor Clinic. JAMA Oncol 2016; 2:922.
  79. Gasparotto D, Rossi S, Campagna D, et al. Imatinib-Sensitizing KIT Mutation in a Carney-Stratakis-Associated GI Stromal Tumor. J Clin Oncol 2016; 34:e99.
  80. 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; 47:853.
  81. Hostein I, Faur N, Primois C, et al. BRAF mutation status in gastrointestinal stromal tumors. Am J Clin Pathol 2010; 133:141.
  82. Janku F, Wheler JJ, Naing A, et al. PIK3CA mutations in advanced cancers: characteristics and outcomes. Oncotarget 2012; 3:1566.
  83. Corless CL. Gastrointestinal stromal tumors: what do we know now? Mod Pathol 2014; 27 Suppl 1:S1.
  84. Shi E, Chmielecki J, Tang CM, et al. FGFR1 and NTRK3 actionable alterations in "Wild-Type" gastrointestinal stromal tumors. J Transl Med 2016; 14:339.
  85. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006; 23:70.
  86. Huang HY, Li CF, Huang WW, et al. A modification of NIH consensus criteria to better distinguish the highly lethal subset of primary localized gastrointestinal stromal tumors: a subdivision of the original high-risk group on the basis of outcome. Surgery 2007; 141:748.
  87. Chun HJ, Byun JY, Chun KA, et al. Gastrointestinal leiomyoma and leiomyosarcoma: CT differentiation. J Comput Assist Tomogr 1998; 22:69.
  88. Levy AD, Remotti HE, Thompson WM, et al. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics 2003; 23:283.
  89. Ghanem N, Altehoefer C, Furtwängler A, et al. Computed tomography in gastrointestinal stromal tumors. Eur Radiol 2003; 13:1669.
  90. Burkill GJ, Badran M, Al-Muderis O, et al. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread. Radiology 2003; 226:527.
  91. Hatch GF 3rd, Wertheimer-Hatch L, Hatch KF, et al. Tumors of the esophagus. World J Surg 2000; 24:401.
  92. Miettinen M, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. Am J Surg Pathol 2005; 29:52.
  93. Miettinen M, Makhlouf H, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up. Am J Surg Pathol 2006; 30:477.
  94. Emory TS, Sobin LH, Lukes L, et al. Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol 1999; 23:82.
  95. Giuliano K, Nagarajan N, Canner J, et al. Gastric and small intestine gastrointestinal stromal tumors: Do outcomes differ? J Surg Oncol 2017; 115:351.
  96. 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; 13:265.
  97. Kukar M, Kapil A, Papenfuss W, et al. Gastrointestinal stromal tumors (GISTs) at uncommon locations: a large population based analysis. J Surg Oncol 2015; 111:696.
  98. Güller U, Tarantino I, Cerny T, et al. Population-based SEER trend analysis of overall and cancer-specific survival in 5138 patients with gastrointestinal stromal tumor. BMC Cancer 2015; 15:557.
  99. Hohenberger P, Ronellenfitsch U, Oladeji O, et al. Pattern of recurrence in patients with ruptured primary gastrointestinal stromal tumour. Br J Surg 2010; 97:1854.
  100. Hølmebakk T, Bjerkehagen B, Boye K, et al. Definition and clinical significance of tumour rupture in gastrointestinal stromal tumours of the small intestine. Br J Surg 2016; 103:684.
  101. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol 2008; 39:1411.
  102. Taniguchi M, Nishida T, Hirota S, et al. Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors. Cancer Res 1999; 59:4297.
  103. Singer S, Rubin BP, Lux ML, et al. Prognostic value of KIT mutation type, mitotic activity, and histologic subtype in gastrointestinal stromal tumors. J Clin Oncol 2002; 20:3898.
  104. Kim TW, Lee H, Kang YK, et al. Prognostic significance of c-kit mutation in localized gastrointestinal stromal tumors. Clin Cancer Res 2004; 10:3076.
  105. Antonescu CR, Sommer G, Sarran L, et al. Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clin Cancer Res 2003; 9:3329.
  106. Wardelmann E, Losen I, Hans V, et al. Deletion of Trp-557 and Lys-558 in the juxtamembrane domain of the c-kit protooncogene is associated with metastatic behavior of gastrointestinal stromal tumors. Int J Cancer 2003; 106:887.
  107. Bachet JB, Hostein I, Le Cesne A, et al. Prognosis and predictive value of KIT exon 11 deletion in GISTs. Br J Cancer 2009; 101:7.
  108. Wozniak A, Rutkowski P, Piskorz A, et al. Prognostic value of KIT/PDGFRA mutations in gastrointestinal stromal tumours (GIST): Polish Clinical GIST Registry experience. Ann Oncol 2012; 23:353.
  109. Joensuu H, Rutkowski P, Nishida T, et al. KIT and PDGFRA mutations and the risk of GI stromal tumor recurrence. J Clin Oncol 2015; 33:634.
  110. DeMatteo RP, Maki RG, Agulnik M, et al.. Gastrointestinal Stromal Tumor. In: AJCC Cancer Staging Manual, 8th, Amin MB (Ed), AJCC, Chicago 2017. p.523.
  111. Gold JS, Gönen M, Gutiérrez A, et al. Development and validation of a prognostic nomogram for recurrence-free survival after complete surgical resection of localised primary gastrointestinal stromal tumour: a retrospective analysis. Lancet Oncol 2009; 10:1045.
  112. Bischof DA, Kim Y, Behman R, et al. A nomogram to predict disease-free survival after surgical resection of GIST. J Gastrointest Surg 2014; 18:2123.
  113. Rossi S, Miceli R, Messerini L, et al. Natural history of imatinib-naive GISTs: a retrospective analysis of 929 cases with long-term follow-up and development of a survival nomogram based on mitotic index and size as continuous variables. Am J Surg Pathol 2011; 35:1646.
  114. DeMatteo RP, Lewis JJ, Leung D, et al. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000; 231:51.
  115. Liegl B, Hornick JL, Lazar AJ. Contemporary pathology of gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009; 23:49.
  116. Maynard MA, Marino-Enriquez A, Fletcher JA, et al. Thyroid hormone inactivation in gastrointestinal stromal tumors. N Engl J Med 2014; 370:1327.
  117. Gasparotto D, Rossi S, Bearzi I, et al. Multiple primary sporadic gastrointestinal stromal tumors in the adult: an underestimated entity. Clin Cancer Res 2008; 14:5715.
  118. Tio TL, Tytgat GN, den Hartog Jager FC. Endoscopic ultrasonography for the evaluation of smooth muscle tumors in the upper gastrointestinal tract: an experience with 42 cases. Gastrointest Endosc 1990; 36:342.
  119. National Comprehensive Cancer Network (NCCN). NCCN Clinical practice guidelines in oncology. http://www.nccn.org/professionals/physician_gls/f_guidelines.asp (Accessed on February 27, 2016).
  120. Eriksson M, Reichardt P, Sundby Hall K, et al. Needle biopsy through the abdominal wall for the diagnosis of gastrointestinal stromal tumour - Does it increase the risk for tumour cell seeding and recurrence? Eur J Cancer 2016; 59:128.
  121. Watson RR, Binmoeller KF, Hamerski CM, et al. Yield and performance characteristics of endoscopic ultrasound-guided fine needle aspiration for diagnosing upper GI tract stromal tumors. Dig Dis Sci 2011; 56:1757.
  122. Demetri GD, Benjamin RS, Blanke CD, et al. NCCN task force report: optimal management of patients with gastrointestinal stromal tumor (GIST)- Update of NCCN Clinical Practice Guidelines. J Natl Comp Cancer Net 2007; 5(2 suppl):S.
  123. Gayed I, Vu T, Iyer R, et al. The role of 18F-FDG PET in staging and early prediction of response to therapy of recurrent gastrointestinal stromal tumors. J Nucl Med 2004; 45:17.
  124. Kamiyama Y, Aihara R, Nakabayashi T, et al. 18F-fluorodeoxyglucose positron emission tomography: useful technique for predicting malignant potential of gastrointestinal stromal tumors. World J Surg 2005; 29:1429.
  125. Lee SH, Ha HK, Byun JY, et al. Radiological features of leiomyomatous tumors of the colon and rectum. J Comput Assist Tomogr 2000; 24:407.
  126. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases. Am J Surg Pathol 2000; 24:1339.