Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Epidemiology, classification, clinical presentation, prognostic features, and diagnostic work-up of gastrointestinal mesenchymal neoplasms including GIST

Jeffrey Morgan, MD
Chandrajit P Raut, MD, MSc, FACS
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 (GI) tract typically present as subepithelial neoplasms, and they are divided broadly into two groups. The most common group consists of neoplasms that are collectively referred to as gastrointestinal stromal tumors (GISTs). They are most often located in the stomach and proximal small intestine, but can occur in any portion of the alimentary tract including occasionally in the omentum, mesentery, and peritoneum [1-5]. The current view is that the overwhelming majority of mesenchymal tumors arising in the GI tract fall into the GIST category, and they are identified mainly by expression of KIT protein; as a group, these tumors are more specifically defined by the presence of activating mutations in the KIT or platelet-derived growth factor receptor A (PDGFRA) genes.

A far less common group of mesenchymal GI 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, diagnostic workup of localized GISTs will be reviewed here. Treatment for GISTs, and GI tract leiomyomas and leiomyosarcomas are discussed elsewhere. (See "Local treatment for gastrointestinal stromal tumors, leiomyomas, and leiomyosarcomas of the gastrointestinal tract" and "Tyrosine kinase inhibitor therapy for advanced gastrointestinal stromal tumors" and "Adjuvant and neoadjuvant imatinib for gastrointestinal stromal tumors".)


Despite being the most common nonepithelial benign neoplasm involving the GI tract, mesenchymal tumors are thought to constitute only 1 percent of primary GI cancers [2,3]. However, their true frequency is unknown. Epidemiologic data such as that from National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program are difficult to interpret since the early definition of so-called "malignant GIST" was derived from criteria published in 1990, at a time before GIST was molecularly characterized [7].

Experience from epidemiologic studies and active GIST therapeutic trials suggest that the annual incidence of GIST in the United States is at least 4000 to 6000 new cases (roughly 7 to 20 cases per million population per year) [7-10]. The most reliable international epidemiologic data are derived from population-based studies that reexamined histologically and immunohistochemically all cases of potential GIST diagnosed between 1983 and 2000 within one province of Sweden [11], between 1990 and 2003 in all of Iceland [12], between 1995 and 2003 in the Netherlands [13], and between 1998 and 2004 in Taiwan [14]. In the Swedish study, 288 of the 1460 cases examined were felt to represent GISTs, for an annual incidence of 14.5 per million population. The Icelandic study reported an incidence of 11 per million population [12].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Sep 2017. | This topic last updated: Feb 24, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  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. Kawanowa K, Sakuma Y, Sakurai S, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol 2006; 37:1527.
  16. 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.
  17. 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.
  18. 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.
  19. Wang JH, Lasota J, Miettinen M. Succinate Dehydrogenase Subunit B (SDHB) Is Expressed in Neurofibromatosis 1-Associated Gastrointestinal Stromal Tumors (Gists): Implications for the SDHB Expression Based Classification of Gists. J Cancer 2011; 2:90.
  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. Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol 2001; 54:96.
  22. 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.
  23. 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.
  24. Heinrich MC, Corless CL, Duensing A, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science 2003; 299:708.
  25. 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.
  26. Bachet JB, Landi B, Laurent-Puig P, et al. Diagnosis, prognosis and treatment of patients with gastrointestinal stromal tumour (GIST) and germline mutation of KIT exon 13. Eur J Cancer 2013; 49:2531.
  27. Forde PM, Cochran RL, Boikos SA, et al. Familial GI Stromal Tumor With Loss of Heterozygosity and Amplification of Mutant KIT. J Clin Oncol 2016; 34:e13.
  28. Broudy VC. Stem cell factor and hematopoiesis. Blood 1997; 90:1345.
  29. 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.
  30. 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.
  31. Pappo AS, Janeway KA. Pediatric gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009; 23:15.
  32. 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.
  33. 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.
  34. Janeway KA, Pappo A. Treatment guidelines for gastrointestinal stromal tumors in children and young adults. J Pediatr Hematol Oncol 2012; 34 Suppl 2:S69.
  35. 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.
  36. Agaram NP, Laquaglia MP, Ustun B, et al. Molecular characterization of pediatric gastrointestinal stromal tumors. Clin Cancer Res 2008; 14:3204.
  37. 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.
  38. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Int J Surg Pathol 2002; 10:81.
  39. Newman PL, Wadden C, Fletcher CD. Gastrointestinal stromal tumours: correlation of immunophenotype with clinicopathological features. J Pathol 1991; 164:107.
  40. 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.
  41. Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279:577.
  42. Lee JR, Joshi V, Griffin JW Jr, et al. Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular identity with gastrointestinal stromal tumor. Am J Surg Pathol 2001; 25:979.
  43. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006; 23:70.
  44. 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.
  45. Lux ML, Rubin BP, Biase TL, et al. KIT extracellular and kinase domain mutations in gastrointestinal stromal tumors. Am J Pathol 2000; 156:791.
  46. Rubin BP, Singer S, Tsao C, et al. KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res 2001; 61:8118.
  47. Emile JF, Théou N, Tabone S, et al. Clinicopathologic, phenotypic, and genotypic characteristics of gastrointestinal mesenchymal tumors. Clin Gastroenterol Hepatol 2004; 2:597.
  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. 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.
  50. 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.
  51. 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.
  52. 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.
  53. 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.
  54. Killian JK, Miettinen M, Walker RL, et al. Recurrent epimutation of SDHC in gastrointestinal stromal tumors. Sci Transl Med 2014; 6:268ra177.
  55. Pantaleo MA, Astolfi A, Indio V, et al. SDHA loss-of-function mutations in KIT-PDGFRA wild-type gastrointestinal stromal tumors identified by massively parallel sequencing. J Natl Cancer Inst 2011; 103:983.
  56. 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.
  57. 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.
  58. Wang L, Vargas H, French SW. Cellular origin of gastrointestinal stromal tumors: a study of 27 cases. Arch Pathol Lab Med 2000; 124:1471.
  59. 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.
  60. 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.
  61. 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.
  62. 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.
  63. 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.
  64. Min KW. Small intestinal stromal tumors with skeinoid fibers. Clinicopathological, immunohistochemical, and ultrastructural investigations. Am J Surg Pathol 1992; 16:145.
  65. Miettinen M, Kopczynski J, Makhlouf HR, et al. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases. Am J Surg Pathol 2003; 27:625.
  66. 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.
  67. 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.
  68. Lee JS, Nascimento AG, Farnell MB, et al. Epithelioid gastric stromal tumors (leiomyoblastomas): a study of fifty-five cases. Surgery 1995; 118:653.
  69. Chun HJ, Byun JY, Chun KA, et al. Gastrointestinal leiomyoma and leiomyosarcoma: CT differentiation. J Comput Assist Tomogr 1998; 22:69.
  70. Levy AD, Remotti HE, Thompson WM, et al. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics 2003; 23:283.
  71. Ghanem N, Altehoefer C, Furtwängler A, et al. Computed tomography in gastrointestinal stromal tumors. Eur Radiol 2003; 13:1669.
  72. 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.
  73. Hatch GF 3rd, Wertheimer-Hatch L, Hatch KF, et al. Tumors of the esophagus. World J Surg 2000; 24:401.
  74. Connolly EM, Gaffney E, Reynolds JV. Gastrointestinal stromal tumours. Br J Surg 2003; 90:1178.
  75. 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.
  76. 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.
  77. 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.
  78. 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.
  79. 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; 32:1563.
  80. American Joint Committee on Cancer. American Joint Committee on Cancer Staging Manual, 7th, Edge SB, Byrd DR, Compton CC, et al (Eds), Springer, New York 2010. p.175.
  81. 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.
  82. 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.
  83. Rutkowski P, Nowecki ZI, Michej W, et al. Risk criteria and prognostic factors for predicting recurrences after resection of primary gastrointestinal stromal tumor. Ann Surg Oncol 2007; 14:2018.
  84. 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.
  85. 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.
  86. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol 2008; 39:1411.
  87. Taniguchi M, Nishida T, Hirota S, et al. Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors. Cancer Res 1999; 59:4297.
  88. 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.
  89. 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.
  90. 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.
  91. 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.
  92. 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.
  93. 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.
  94. Wang HC, Li TY, Chao YJ, et al. KIT Exon 11 Codons 557-558 Deletion Mutation Promotes Liver Metastasis Through the CXCL12/CXCR4 Axis in Gastrointestinal Stromal Tumors. Clin Cancer Res 2016; 22:3477.
  95. 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.
  96. Yamaguchi U, Nakayama R, Honda K, et al. Distinct gene expression-defined classes of gastrointestinal stromal tumor. J Clin Oncol 2008; 26:4100.
  97. Hill MA, Gong C, Casey TJ, et al. Detection of K-ras mutations in resected primary leiomyosarcoma. Cancer Epidemiol Biomarkers Prev 1997; 6:1095.
  98. Chou FF, Eng HL, Sheen-Chen SM. Smooth muscle tumors of the gastrointestinal tract: analysis of prognostic factors. Surgery 1996; 119:171.
  99. Pink D, Schoeler D, Lindner T, et al. Severe hypoglycemia caused by paraneoplastic production of IGF-II in patients with advanced gastrointestinal stromal tumors: a report of two cases. J Clin Oncol 2005; 23:6809.
  100. Liegl B, Hornick JL, Lazar AJ. Contemporary pathology of gastrointestinal stromal tumors. Hematol Oncol Clin North Am 2009; 23:49.
  101. 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.
  102. 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.
  103. Maynard MA, Marino-Enriquez A, Fletcher JA, et al. Thyroid hormone inactivation in gastrointestinal stromal tumors. N Engl J Med 2014; 370:1327.
  104. 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.
  105. 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.
  106. Yasuda K, Cho E, Nakajima M, Kawai K. Diagnosis of submucosal lesions of the upper gastrointestinal tract by endoscopic ultrasonography. Gastrointest Endosc 1990; 36:S17.
  107. Boyce GA, Sivak MV Jr, Rösch T, et al. Evaluation of submucosal upper gastrointestinal tract lesions by endoscopic ultrasound. Gastrointest Endosc 1991; 37:449.
  108. Palazzo L, Landi B, Cellier C, et al. Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours. Gut 2000; 46:88.
  109. 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.
  110. 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).
  111. 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.
  112. 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.
  113. 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.
  114. 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.
  115. Van den Abbeele AD. The lessons of GIST--PET and PET/CT: a new paradigm for imaging. Oncologist 2008; 13 Suppl 2:8.