Medication Summary

The goals of pharmacotherapy in patients with gastrointestinal stromal tumors (GISTs) are to induce remission, reduce morbidity, and prevent complications. The tyrosine kinase inhibitors (TKIs) imatinib or avapritinib are indicated for unresectable GISTs and metastatic malignant GISTs. Evidence of genetic markers for KIT and PDGFRA are essential to determine which therapeutic options are optimal.

Class Summary

Agents with strong tyrosine-kinase inhibition activity of the bcr-abl abnormality in all cell-cycle phases of gastric tumor cells.

Imatinib mesylate (Gleevec)

View full drug information

Specifically designed to inhibit tyrosine-kinase activity of the bcr-abl kinase in GI stromal tumors. These tumors are characterized by expression of the product of the proto-oncogene c-kit and often harbor gain-of-function KIT mutations, leading to ligand-independent kinase activation. Gleevec inhibits ABL, KIT, and PDGFR tyrosine kinase.

Sunitinib malate (SU-11248, Sutent)

View full drug information

Multikinase inhibitor that targets several tyrosine-kinase inhibitors implicated in tumor growth, pathologic angiogenesis, and metastatic progression. Inhibits platelet-derived growth factor receptors (ie, PDGFR-alpha, PDGFR-beta), vascular endothelial growth factor receptors (ie, VEGFR1, VEGFR2, VEGFR3), stem cell factor receptor (KIT), Fms-like tyrosine kinase-3 (FLT3), colony-stimulating factor receptor type 1 (CSF-1R), and the glial cell-line–derived neurotrophic factor receptor (RET).

Indicated for persons with GISTs whose disease has progressed or who are unable to tolerate treatment with imatinib (Gleevec). Delays median time to tumor progression.

Regorafenib (Stivarga)

View full drug information

Regorafenib is a tyrosine kinase inhibitor. It is indicated for locally advanced, unresectable gastrointestinal stromal tumors that no longer respond to other treatments (eg, imatinib, sunitinib).

Avapritinib (Ayvakit)

View full drug information

Avapritinib is a tyrosine kinase inhibitor that targets PDGFRA and PDGFRA D842 mutants as well as multiple KIT exon 11, 11/17 and 17 mutants. This results in the inhibition of PDGFRa- and c-Kit-mediated signal transduction pathways and the inhibition of proliferation in tumor cells that express these PDGFRa and c-Kit mutants. It is indicated for adults with unresectable or metastatic GIST harboring a platelet-derived growth factor receptor alpha (PDGFRA) exon 18 mutations, including PDGFRA D842V mutations.

Ripretinib (Qinlock)

View full drug information

Ripretinib is a broad-spectrum inhibitor or proto-oncogene receptor tyrosine kinase (KIT) and platelet derived growth factor receptor-A (PDGFRA), including wild type, primary, and secondary mutations. It is indicated for advanced GISTs in adults who have received prior treatment with 3 or more kinase inhibitors, including imatinib.

PDQ Adult Treatment Editorial Board. Gastrointestinal Stromal Tumors Treatment (Adult) (PDQ®): Health Professional Version. September 16, 2020. [QxMD MEDLINE Link]. [Full Text].
Ghanem N, Altehoefer C, Furtwängler A, Winterer J, Schäfer O, Springer O, et al. Computed tomography in gastrointestinal stromal tumors. Eur Radiol. 2003 Jul. 13 (7):1669-78. [QxMD MEDLINE Link].
Zhou HY, Zhang XM, Zeng NL, Jian SH, Tang W. Use of conventional MR imaging and diffusion-weighted imaging for evaluating the risk grade of gastrointestinal stromal tumors. J Magn Reson Imaging. 2012 Dec. 36 (6):1395-401. [QxMD MEDLINE Link].
Chak A, Canto MI, Rosch T, et al. Endosonographic differentiation of benign and malignant stromal cell tumors. Gastrointest Endosc. 1997 Jun. 45(6):468-73. [QxMD MEDLINE Link].
DeMatteo RP, Ballman KV, Antonescu CR, et al. Adjuvant imatinib mesylate after resection of primary gastrointestinal stromal tumour: a randomized, double-blind, placebo-controlled trial. Lancet. March 28, 2009. 373:1097-1104. [QxMD MEDLINE Link].
Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998 May. 152 (5):1259-69. [QxMD MEDLINE Link]. [Full Text].
Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998 Jan 23. 279 (5350):577-80. [QxMD MEDLINE Link].
Kang HJ, Koh KH, Yang E, You KT, Kim HJ, Paik YK. Differentially expressed proteins in gastrointestinal stromal tumors with KIT and PDGFRA mutations. Proteomics. 2006 Feb. 6(4):1151-7. [QxMD MEDLINE Link].
Medeiros F, Corless CL, Duensing A, Hornick JL, Oliveira AM, Heinrich MC. KIT-negative gastrointestinal stromal tumors: proof of concept and therapeutic implications. Am J Surg Pathol. 2004 Jul. 28(7):889-94. [QxMD MEDLINE Link].
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 Oct. 13(10):1134-42. [QxMD MEDLINE Link].
Van Oosterom AT, Judson I, Verweij J, et al. STI 571, an active drug in metastatic gastrointestinal tumors (GIST), AN EORTC phase I study. Plenary Presentation. The American Society of Clinical Oncology, 37th Annual Meeting. 2001.
Bauer S, Lang H, Schütte J, Hartmann JT. Complete remission with imatinib in metastastic gastrointestinal stromal tumors. J Clin Oncol. 2005 Sep 20. 23(27):6800-1; author reply 6801-2. [QxMD MEDLINE Link].
Benjamin RS, Blanke CD, Blay JY, Bonvalot S, Eisenberg B. Management of gastrointestinal stromal tumors in the imatinib era: selected case studies. Oncologist. 2006 Jan. 11(1):9-20. [QxMD MEDLINE Link].
Koh JS, Trent J, Chen L, et al. Gastrointestinal stromal tumors: overview of pathologic features, molecular biology, and therapy with imatinib mesylate. Histol Histopathol. 2004 Apr. 19(2):565-74. [QxMD MEDLINE Link].
Maki RG. Gastrointestinal Stromal Tumors Respond to Tyrosine Kinase-targeted Therapy. Curr Treat Options Gastroenterol. 2004 Feb. 7(1):13-17. [QxMD MEDLINE Link].
Melichar B, Voboril Z, Nozicka J, Ryska A, Urminská H, Vanecek T. Pathological complete response in advanced gastrointestinal stromal tumor after imatinib therapy. Intern Med. 2005 Nov. 44(11):1163-8. [QxMD MEDLINE Link].
Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001 Jan. 438(1):1-12. [QxMD MEDLINE Link].
Miettinen M, El-Rifai W, H L Sobin L, Lasota J. Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol. 2002 May. 33 (5):478-83. [QxMD MEDLINE Link].
Fukasawa T, Chong JM, Sakurai S, Koshiishi N, Ikeno R, Tanaka A, et al. Allelic loss of 14q and 22q, NF2 mutation, and genetic instability occur independently of c-kit mutation in gastrointestinal stromal tumor. Jpn J Cancer Res. 2000 Dec. 91 (12):1241-9. [QxMD MEDLINE Link].
Rege TA, Wagner AJ, Corless CL, Heinrich MC, Hornick JL. "Pediatric-type" gastrointestinal stromal tumors in adults: distinctive histology predicts genotype and clinical behavior. Am J Surg Pathol. 2011 Apr. 35(4):495-504. [QxMD MEDLINE Link].
Hirota S, Isozaki K. Pathology of gastrointestinal stromal tumors. Pathol Int. 2006 Jan. 56(1):1-9. [QxMD MEDLINE Link].
Rubin BP, Singer S, Tsao C, Duensing A, Lux ML, Ruiz R, et al. KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res. 2001 Nov 15. 61 (22):8118-21. [QxMD MEDLINE Link].
Agaimy A, Terracciano LM, Dirnhofer S, Tornillo L, Foerster A, Hartmann A, et al. V600E BRAF mutations are alternative early molecular events in a subset of KIT/PDGFRA wild-type gastrointestinal stromal tumours. J Clin Pathol. 2009 Jul. 62 (7):613-6. [QxMD MEDLINE Link].
Fukasawa T, Chong JM, Sakurai S, Koshiishi N, Ikeno R, Tanaka A, et al. Allelic loss of 14q and 22q, NF2 mutation, and genetic instability occur independently of c-kit mutation in gastrointestinal stromal tumor. Jpn J Cancer Res. 2000 Dec. 91 (12):1241-9. [QxMD MEDLINE Link].
[Guideline] Casli PG, et a; ESMO Guidelines Committee, EURACAN and GENTURIS. Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2022 Jan. 33 (1):20-33. [QxMD MEDLINE Link]. [Full Text].
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 Jun. 74 (6):543-52. [QxMD MEDLINE Link].
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 Jan. 24 (1):298-302. [QxMD MEDLINE Link]. [Full Text].
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 Nov 1. 117 (2):289-93. [QxMD MEDLINE Link].
Goettsch WG, Bos SD, Breekveldt-Postma N, Casparie M, Herings RM, Hogendoorn PC. Incidence of gastrointestinal stromal tumours is underestimated: results of a nation-wide study. Eur J Cancer. 2005 Dec. 41 (18):2868-72. [QxMD MEDLINE Link].
Rubió J, Marcos-Gragera R, Ortiz MR, Miró J, Vilardell L, Gironès J, et al. Population-based incidence and survival of gastrointestinal stromal tumours (GIST) in Girona, Spain. Eur J Cancer. 2007 Jan. 43 (1):144-8. [QxMD MEDLINE Link].
Nilsson B, Bümming P, Meis-Kindblom JM, Odén A, Dortok A, Gustavsson B, 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 Feb 15. 103 (4):821-9. [QxMD MEDLINE Link].
Aparicio T, Boige V, Sabourin JC, Crenn P, Ducreux M, Le Cesne A. Prognostic factors after surgery of primary resectable gastrointestinal stromal tumours. Eur J Surg Oncol. 2004 Dec. 30(10):1098-103. [QxMD MEDLINE Link].
Koga H, Ochiai A, Nakanishi Y, et al. Reevaluation of prognostic factors in gastric leiomyosarcoma. Am J Gastroenterol. 1995 Aug. 90(8):1307-12. [QxMD MEDLINE Link].
Ng EH, Pollock RE, Romsdahl MM. Prognostic implications of patterns of failure for gastrointestinal leiomyosarcomas. Cancer. 1992 Mar 15. 69(6):1334-41. [QxMD MEDLINE Link].
Rosen MJ, Heniford BT. Endoluminal gastric surgery: the modern era of minimally invasive surgery. Surg Clin North Am. 2005 Oct. 85(5):989-1007, vii. [QxMD MEDLINE Link].
Piessen G, Lefèvre JH, Cabau M, et al. Laparoscopic Versus Open Surgery for Gastric Gastrointestinal Stromal Tumors: What Is the Impact on Postoperative Outcome and Oncologic Results?. Ann Surg. 2015 Nov. 262 (5):831-40. [QxMD MEDLINE Link].
Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol. 2002 May. 33 (5):459-65. [QxMD MEDLINE Link].
Gold JS, Gönen M, Gutiérrez A, Broto JM, García-del-Muro X, Smyrk TC, 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 Nov. 10 (11):1045-52. [QxMD MEDLINE Link].
[Guideline] National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Gastrointestinal Stromal Tumors (GISTs). NCCN.org. Available at https://www.nccn.org/professionals/physician_gls/pdf/gist.pdf. Version 1.2022 — January 21, 2022; Accessed: May 23, 2022.
Sineshaw HM, Jemal A, Lin CC, McGinnis LS, Ward EM. Contemporary Patterns and Survival Outcome of Adjuvant Systemic Therapy for Localized Gastrointestinal Stromal Tumors. Am J Clin Oncol. 2015 Jan 16. [QxMD MEDLINE Link].
Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol. 2001 Feb. 54(2):96-102. [QxMD MEDLINE Link].
King DM. The radiology of gastrointestinal stromal tumours (GIST). Cancer Imaging. 2005. 5:150-6. [QxMD MEDLINE Link].
Yamashita F, Sasatomi E, Kiyama M, Fukumori K, Yano Y, Kato O, et al. Radiographic observation of a case of gastrointestinal stromal tumor in stomach. Kurume Med J. 2001. 48 (3):233-6. [QxMD MEDLINE Link].
Stroobants S, Goeminne J, Seegers M, Dimitrijevic S, Dupont P, Nuyts J, et al. 18FDG-Positron emission tomography for the early prediction of response in advanced soft tissue sarcoma treated with imatinib mesylate (Glivec). Eur J Cancer. 2003 Sep. 39 (14):2012-20. [QxMD MEDLINE Link].
Palazzo L, Landi B, Cellier C, et al. Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours. Gut. 2000 Jan. 46(1):88-92. [QxMD MEDLINE Link].
Fields S, Libson E. CT-guided aspiration core needle biopsy of gastrointestinal wall lesions. J Comput Assist Tomogr. 2000 Mar-Apr. 24(2):224-8. [QxMD MEDLINE Link].
Kikuchi H, Yamashita K, Kawabata T, Yamamoto M, Hiramatsu Y, Kondo K. Immunohistochemical and genetic features of gastric and metastatic liver gastrointestinal stromal tumors: sequential analyses. Cancer Sci. 2006 Feb. 97(2):127-32. [QxMD MEDLINE Link].
Montgomery E, Abraham SC, Fisher C, et al. CD44 loss in gastric stromal tumors as a prognostic marker. Am J Surg Pathol. 2004 Feb. 28(2):168-77. [QxMD MEDLINE Link].
Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol. 1995 Jan. 103(1):41-7. [QxMD MEDLINE Link].
Tzen CY, Mau BL. Analysis of CD117-negative gastrointestinal stromal tumors. World J Gastroenterol. 2005 Feb 21. 11(7):1052-5. [QxMD MEDLINE Link].
Soft Tissue Sarcoma. Amin MB, Edge S, Greene F. AJCC Cancer Staging Manual. 8th Ed. Cham: Switzerland: Springer International Publishing; 2017. 489-548.
Joensuu H, Roberts PJ, Sarlomo-Rikala M, et al. Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med. 2001 Apr 5. 344(14):1052-6. [QxMD MEDLINE Link].
Tuveson DA, Willis NA, Jacks T, et al. STI571 inactivation of the gastrointestinal stromal tumor c-KIT oncoprotein: biological and clinical implications. Oncogene. 2001 Aug 16. 20(36):5054-8. [QxMD MEDLINE Link].
Pisters PW, Patel SR. Gastrointestinal stromal tumors: current management. J Surg Oncol. 2010 Oct 1. 102 (5):530-8. [QxMD MEDLINE Link].
Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006 Oct 14. 368 (9544):1329-38. [QxMD MEDLINE Link].
Senchak J, Ahr K, von Mehren M. Gastrointestinal Stromal Tumors: What Is the Best Sequence of TKIs?. Curr Treat Options Oncol. 2022 May. 23 (5):749-761. [QxMD MEDLINE Link].
Nannini M, Rizzo A, Indio V, Schipani A, Astolfi A, Pantaleo MA. Targeted therapy in SDH-deficient GIST. Ther Adv Med Oncol. 2021. 13:17588359211023278. [QxMD MEDLINE Link]. [Full Text].
Neppala P, Banerjee S, Fanta PT, Yerba M, Porras KA, Burgoyne AM, et al. Current management of succinate dehydrogenase-deficient gastrointestinal stromal tumors. Cancer Metastasis Rev. 2019 Sep. 38 (3):525-535. [QxMD MEDLINE Link].
Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004 Sep 25-Oct 1. 364(9440):1127-34. [QxMD MEDLINE Link].
Joensuu H, Eriksson M, Hatrmann I, et al. Twelve versus 36 months of adjuvant imatinib (IM) as treatment of operable GIST with a high risk of recurrence: Final results of a randomized trial (SSGXVIII/AIO). 2011 American Society of Clinical Oncology (ASCO) Annual Meeting. Abstract LBA1. Presented June 5, 2011.
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 Jan 26. 381(9863):295-302. [QxMD MEDLINE Link].
Wu L, Zhang Z, Yao H, Liu K, Wen Y, Xiong L. Clinical efficacy of second-generation tyrosine kinase inhibitors in imatinib-resistant gastrointestinal stromal tumors: a meta-analysis of recent clinical trials. Drug Des Devel Ther. 2014. 8:2061-7. [QxMD MEDLINE Link]. [Full Text].
Heinrich M, von Mehren M, Jones RL, Bauer S, Yoon-Koo K, Schöfski P, et al. Avapritinib is highly active and well-tolerated in patients with advanced GIST driven by a diverse variety of oncogenic mutations in KIT and PDGFRA. Presented at Connective Tissue Oncology Society 2018 Annual Meeting. Rome, IT. 2018 Nov 15. [Full Text].
Bauer S, George S, Yoon-Koo K, Tap WD, Zhou T, Picazio N, et al. An open-label, randomized, phase 3 study of avapritinib vs regorafenib in patients with locally advanced metastatic or unresectable gastrointestinal stromal tumors. Presented at the Annual congress of the European Society for Medical Oncology (ESMO). Munich, Germany. 2018 Oct 19-23. [Full Text].
Qinlock (ripretinib) [package insert]. Waltham, MA: Deciphera Pharmaceuticals, LLC. May 2020. Available at [Full Text].
Boni L, Benevento A, Dionigi G, Rovera F, Dionigi R. Surgical resection for gastrointestinal stromal tumors (GIST): experience on 25 patients. World J Surg Oncol. 2005. 3:78. [QxMD MEDLINE Link].
Bucher P, Egger JF, Gervaz P, Ris F, Weintraub D, Villiger P. An audit of surgical management of gastrointestinal stromal tumours (GIST). Eur J Surg Oncol. 2006 Apr. 32(3):310-4. [QxMD MEDLINE Link].
Knoop M, St Friedrichs K, Dierschke J. Surgical management of gastrointestinal stromal tumors of the stomach. Langenbecks Arch Surg. 2000 Apr. 385(3):194-8. [QxMD MEDLINE Link].
Kosmadakis N, Visvardis EE, Kartsaklis P, Tsimara M, Chatziantoniou A, Panopoulos I. The role of surgery in the management of gastrointestinal stromal tumors (GISTs) in the era of imatinib mesylate effectiveness. Surg Oncol. 2005 Aug. 14(2):75-84. [QxMD MEDLINE Link].
Guo Y, Liu J, Wang F, Wang Q, Zheng G, Liu S, et al. The Role of Surgical Resection Following Tyrosine Kinase Inhibitors Treatment in Patients with Advanced Gastrointestinal Stromal Tumors: A Systematic Review and Meta-analysis. J Cancer. 2019. 10 (23):5785-5792. [QxMD MEDLINE Link]. [Full Text].
Katai H, Sasako M, Sano T. Wedge resection of the stomach for gastric leiomyosarcoma. Br J Surg. 1997 Apr. 84(4):560-1. [QxMD MEDLINE Link].
Zhao Y, Pang T, Zhang B, Wang L, Lv Y, Ling T, et al. Retrospective Comparison of Endoscopic Full-Thickness Versus Laparoscopic or Surgical Resection of Small (≤ 5 cm) Gastric Gastrointestinal Stromal Tumors. J Gastrointest Surg. 2019 Dec 10. [QxMD MEDLINE Link].
Eisenberg BL, Judson I. Surgery and imatinib in the management of GIST: emerging approaches to adjuvant and neoadjuvant therapy. Ann Surg Oncol. 2004 May. 11(5):465-75. [QxMD MEDLINE Link].
Rutkowski P, Nowecki Z, Nyckowski P, Dziewirski W, Grzesiakowska U, Nasierowska-Guttmejer A. Surgical treatment of patients with initially inoperable and/or metastatic gastrointestinal stromal tumors (GIST) during therapy with imatinib mesylate. J Surg Oncol. 2006 Mar 15. 93(4):304-11. [QxMD MEDLINE Link].
Chen H, Pruitt A, Nicol TL, et al. Complete hepatic resection of metastases from leiomyosarcoma prolongs survival. J Gastrointest Surg. 1998 Mar-Apr. 2(2):151-5. [QxMD MEDLINE Link].
Chen YH, Liu KH, Yeh CN, Hsu JT, Liu YY, Tsai CY, et al. Laparoscopic resection of gastrointestinal stromal tumors: safe, efficient, and comparable oncologic outcomes. J Laparoendosc Adv Surg Tech A. 2012 Oct. 22 (8):758-63. [QxMD MEDLINE Link].
Aogi K, Hirai T, Mukaida H, et al. Laparoscopic resection of submucosal gastric tumors. Surg Today. 1999. 29(2):102-6. [QxMD MEDLINE Link].
Bedard EL, Mamazza J, Schlachta CM, Poulin EC. Laparoscopic resection of gastrointestinal stromal tumors: not all tumors are created equal. Surg Endosc. 2006 Mar. 20(3):500-3. [QxMD MEDLINE Link].
Hindmarsh A, Koo B, Lewis MP, Rhodes M. Laparoscopic resection of gastric gastrointestinal stromal tumors. Surg Endosc. 2005 Aug. 19(8):1109-12. [QxMD MEDLINE Link].
Kitano S, Shiraishi N. Minimally invasive surgery for gastric tumors. Surg Clin North Am. 2005 Feb. 85(1):151-64, xi. [QxMD MEDLINE Link].
Nguyen SQ, Divino CM, Wang JL, Dikman SH. Laparoscopic management of gastrointestinal stromal tumors. Surg Endosc. 2006 May. 20(5):713-6. [QxMD MEDLINE Link].
Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. World Health Organization Classification of Tumours of Soft Tissue and Bone. 4th Ed. Lyon, France: IARC; 2013.
Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984 Jan 15. 33 (1):37-42. [QxMD MEDLINE Link].
Costa J, Wesley RA, Glatstein E, Rosenberg SA. The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer. 1984 Feb 1. 53 (3):530-41. [QxMD MEDLINE Link].

Media Gallery

Shown here is a gastric gastrointestinal stromal tumor (GIST). This is a gross specimen following partial gastrectomy. Note the submucosal tumor mass with the classic features of central umbilication and ulceration.
CT scan of the abdomen with oral contrast in a 60-year-old woman with a gastric gastrointestinal stromal tumor (GIST). A huge mass with central necrosis is observed originating from the gastric wall and narrowing its lumen. An ulcer crater can be identified within the mass (arrow).
Photomicrograph of gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 40X. Note the solid sheet of spindle cells.
Photomicrograph of gastric gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 400X. This stromal tumor demonstrates spindle cells with epithelioid features.
Photomicrograph of gastrointestinal stromal tumor (GIST) with immunohistochemical staining for CD117. Note the strong positive staining of tumor cells with negative staining of the adjacent vessel. Positive stain for CD117 is diagnostic of GIST.

of 5

Table 1. TNM Classification for Gastrointestinal Stromal Tumors

Primary tumor (T)
TX	Primary tumor cannot be assessed
T0	No evidence of primary tumor
T1	Tumor 2 cm or smaller
T2	Tumor more than 2 cm but not more than 5 cm
T3	Tumor more than 5 but not more than 10 cm
T4	Tumor more than 10 cm in greatest dimension
Regional lymph node (N)
N0	No regional lymph node metastasis
N1	Regional lymph node metastasis
Metastasis (M)
M0	No distant metastasis
M1	Distant metastases

Table 2. Anatomic Stage/Prognostic Groups for Gastric GISTs

Group	Tumor	Node	Metastasis	Mitotic Rate*
Stage IA	T1 or T2	N0	M0	Low
Stage IB	T3	N0	M0	Low
Stage II	T1	N0	M0	High
	T2	N0	M0	High
	T4	N0	M0	Low
Stage IIIA	T3	N0	M0	High
Stage IIIB	T4	N0	M0	High
Stage IV	Any T	N1	M0	Any rate
Stage IV	Any T	Any N	M1	Any rate
*Mitotic rate: Low (≤5 per 5 mm² or per 50 HPF); High (>5 per 5 mm² or per 50 HPF)

Table 3. Anatomic Stage/Prognostic Groups for Small-Intestinal GISTs

Group	Tumor	Node	Metastasis	Mitotic Rate*
Stage I	T1 or T2	N0	M0	Low
Stage II	T3	N0	M0	Low
Stage IIIA	T1	N0	M0	High
Stage IIIA	T4	N0	M0	Low
Stage IIIB	T2	N0	M0	High
	T3	N0	M0	High
	T4	N0	M0	High
Stage IV	Any T	N1	M0	Any Rate
Stage IV	Any T	Any N	M1	Any Rate
*Mitotic rate: Low (≤5 per 5 mm² or per 50 HPF); High (>5 per 5 mm² or per 50 HPF)

Table 4. Malignant Potential of Gastric GISTs (NCCN)

Tumor Size	Mitotic Rate*	Predicted Metastasis Rate
≤2 cm	Any mitotic rate	0%
>2 to 5 cm	≤5	1.9%
>2 to 5 cm	>5	16%
>5 to 10 cm	≤5	3.6%
>5 to 10 cm	>5	55%
>10 cm	≤5	12%
>10 cm	>5	86%
*Number of metastases per 5 mm² or per 50 high-power field

Table 5. Malignant Potential of Non-Gastric GISTs (NCCN)

Tumor Size	Mitotic Rate*	Predicted Metastasis Rate
≤2 cm	≤5	0%
≤2 cm	>5	50-54%
>2 to 5 cm	≤5	1.9-8.5%
>2 to 5 cm	>5	50-73%
>5 to 10 cm	≤5	24%
>5 to 10 cm	>5	85%
>10 cm	≤5	34-52%
>10 cm	>5	71-90%
*Number of metastases per 5 mm² or per 50 high-power field

Table 4. Staging of Gastric Gastrointestinal Stromal Tumors

Stage	T	N	M	Grade
IA	T1 or T2	N0	M0	Low
IB	T3	N0	M0	Low
II	T1	N0	M0	High
	T2	N0	M0	High
	T4	N0	M0	Low
IIIA	T3	N0	M0	High
IIIB	T4	N0	M0	High
IV	Any T	N1	M0	Any grade
IV	Any T	Any N	M1	Any grade